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Abnormal blink reflex (BR) results mainly from the dysfunction of reticular brainstem pathways and is one of the features of degenerative brain disorders. We aimed to investigate whether patients with Wilson’s disease (WD) have abnormal BR. This was a prospective, observational, single-center study. BR was assessed in accordance with generally accepted standards in 44 newly diagnosed treatment-naïve and 66 treated patients with WD. Any abnormal parameters in BR were observed in 45.5% treatment-naïve patients and 37.9% treated patients (p = 0.429). We also did not observe significant differences in BR parameters and frequency of abnormal findings between treated and treatment naïve patients. Abnormal findings in any of the BR parameters were more frequent in patients with neurological vs. non-neurological presentation (57.5 vs. 28.6%, p = 0.002), present vs. absent Kayser–Fleischer ring (73 vs. 21.5%, p p = 0.009). In addition, longer median R1 and R2 latencies, both ipsilateral and contralateral, were significantly more frequent in neurological than non-neurological WD patients, those with Kayser–Fleischer rings, and those with abnormal MRI findings typical of WD. Our results confirm frequent BR abnormalities in WD, which may be explained by the pathological influence of copper deposits in the circuit linking the basal ganglia, cerebellum and brainstem.

Aim of the work To study the frequency of cranial and peripheral neuropathies in systemic lupus erythematosus (SLE), their clinical characteristics, electrophysiological pattern and relation to disease activity. Patients and methods The study included 30 SLE patients and 20 matched healthy controls. Electrophysiological assessment included routine nerve conduction studies for assessment of peripheral nerves and visual evoked potential, blink reflex, and brain stem auditory evoked potential for assessment of the second, fifth, seventh, and eighth cranial nerves, respectively. Safety of Estrogens in Lupus Erythematosus National Assessment–SLE Disease Activity Index (SELENA–SLEDAI) was assessed. Results The mean age of the patients was 35.8 ± 8.6 years, 27 females and 3 males (9:1) with median disease duration 4.5(1.2–8) years and SELENA-SLEDAI of 12.09 ± 4.94. The mean complement-3 (C3) level was 81.2 ± 26.9 mg/dl and C4 was 11.5 ± 4.4 mg/dl. Peripheral neuropathy was detected electrophysiologically in 66.7% and clinically in 53.3% of the patients and was sensory more than motor. Sensory neuropathy was found in 9(30%), sensorimotor in 11(36.7%), demyelinating in 9(30%), axonal in 6(20%) and both axonal and demyelinating in 5(16.7%) patients. 13.3% had subclinical neuropathy. Sensorimotor and sensory neuropathies were detected in 36.7% and 30% of patients, respectively. Cranial neuropathy was not detected in any patient or control clinically or electrophysiologically. Peripheral neuropathy significantly correlated with SELENA-SLEDAI (r = 0.55, p = 0.002) and negatively with C3 and C4 (r = −0.65, p = 0.012 and r = −0.63, p = 0.015 respectively). Conclusion Peripheral neuropathy is a well-recognized but underestimated manifestation of neuropsychiatric SLE with predominance of sensorimotor variant. Peripheral neuropathy is associated with disease activity and complement consumption.

Objective To investigate the effects on the blink reflex (BR) of single stimuli applied to the pedunculopontine tegmental nucleus (PPTg). Methods The BR was evoked by stimulating the supraorbital nerve (SON) in fifteen patients suffering from idiopathic Parkinson’s disease (PD) who had electrodes monolaterally or bilaterally implanted in the PPTg for deep brain stimulation (DBS). Single stimuli were delivered to the PPTg through externalized electrode connection wires 3–4 days following PPTg implantation. Results PPTg stimuli increased the latency and reduced duration, amplitude and area of the R2 component of the BR in comparison to the response recorded in the absence of PPTg stimulation. These effects were independent of the side of SON stimulation and were stable for interstimulus interval (ISI) between PPTg prepulse and SON stimulus from 0 to 110 ms. The PPTg-induced prepulse inhibition of the BR was bilaterally present in the brainstem. The R1 component was unaffected. Conclusions The prepulse inhibition of the R2 component may be modulated by the PPTg. Significance These findings suggest that abnormalities of BR occurring in PD may be ascribed to a reduction of basal ganglia-mediated inhibition of brainstem excitability.

Background Ptosis can be a manifestation of a more serious situation. Hence, the analysis of the complaint and the search for etiology are crucial in such cases. Ptosis has many causes; some of them lead to unilateral ptosis while others cause bilateral ptosis. For instance, myasthenia gravis is a cause of bilateral ptosis while oculomotor nerve palsy induces unilateral disease. Proper evaluation of the patient and identification of the cause are important to achieve accurate management and good prognosis. Case presentation A 47-year-old male patient attended the ER complaining of dropping the right eye lid of 2 days’ duration. There was no associated pain or diplopia. On examination, the extraocular muscles’ (EOM) motility was intact, normal pupil and corneal reflexes, and there was swelling of the upper eyelid. Ophthalmological examination revealed normal anterior and posterior chambers as well as the vitreous and retina. The patient had a previous history of traumatic intracranial hemorrhage that was resolved without surgical intervention. He also had diabetes mellitus and hypertension. The patient was transferred to the MRI unit to perform MRI study of the brain and orbit with MRA and IV contrast administration. MRI findings confirmed the diagnosis of LPS myositis, and the patient received medical treatment and improved. Conclusion Proper radiological diagnosis leads to accurate management and achieves rapid recovery and optimal patient care.

Raffaele Migliorini,1 Anna Maria Comberiati,1 Fernanda Pacella,1 Anna Rosy Longo,1 Daniela Messineo,2 Edoardo Trovato Battagliola,1 Mariaelena Malvasi,1 Elena Pacella,1 Loredana Arrico1 1Department of Sense Organs, University Sapienza, Rome, Italy; 2Department of Radiology, Oncology, and Anatomopathological, University Sapienza, Rome, ItalyCorrespondence: Elena PacellaDepartment of Sense Organs, University Sapienza, Policlinico Umberto I, Viale del Policlinico 155, Rome, 00161, ItalyTel +39 336783409Email elena.pacella@uniroma1.itPurpose: Determine the usefulness of ocular motility testing to detect the presence of muscle entrapment.Materials and Methods: Cross-sectional study of patients with symptoms of diplopia secondary to facial trauma. Inclusion criteria: age between 20 and 80 years; symptoms of diplopia following facial trauma; presence of orbital floor fracture confirmed radiologically; presence of muscle entrapment confirmed at the time of surgery; best-corrected visual acuity of 0.6 or more. Exclusion criteria: muscle entrapment visible on computed tomography; candidate for immediate surgical correction; prior history of strabismus surgery. Outcome measures: Abnormal Head Position (AHP), Hirschberg Corneal Reflexes (CR), Cover/Uncover and Alternating Cover Test, Hertel exophthalmometry, Near Point of Convergence (NPC), Kestenbaum Limbus test, Red Filter test, and Hess screen test.Results: Forty-six subjects (38 males, 8 females, mean age 27 ± 3.3 SD years). Pre-operative assessment: forty-six (100%) reported diplopia on the Red Filter test and showed some degree of abnormality on the Hess Screen test. Forty-two (91%) showed AHP. Forty-one (89%) had exophthalmometry values that differed 2 mm or more between the two eyes and insufficient NPC. Thirty-two (69.6%) showed deficits of 3 mm or more on the Kestenbaum Limbus test. Sixteen (35%) had abnormal Hirschberg corneal reflexes. Eleven (24%) demonstrated constant or intermittent strabismus.Conclusion: Ocular motility testing can differentiate non-invasively, pre-operatively, and cost-effectively the presence of muscle entrapment even when this is not visible on computed tomography.Keywords: orbital blow-out fractures, orbital floor fracture, ocular motility, trauma, delayed surgical repair, muscle entrapment, traumatic diplopia

Introduction The aim of this study was to analyze the functions of pedunculopontine nucleus (PPN) in isolated REM sleep behavior disorder (iRBD) and REM sleep without atonia (RSWA) to investigate the role of PPN in dream-enacting motor behaviors in RBD. We evaluated the activity of PPN through the prepulse modulation (PPM) together with other brainstem reflexes to investigate the differences in changes at brainstem. Methods We included nine patients with isolated RSWA and 10 patients with iRBD. For diagnosis, all patients underwent polysomnography. None of the patients had parkinsonism or dementia. We also included 17 healthy participants with similar age and sex. Blink reflex (BR), PPM of BR, recovery excitability of BR, and auditory startle reflex (ASR) were recorded in all participants. Results There was a prepulse inhibition deficit in iRBD and RSWA groups compared to healthy subjects. The BR-R2 recovery at 200 ms interval was also higher in patients with iRBD and RSWA. In ASR recordings, the response probabilities were higher in the RBD group compared to RSWA and control groups. Conclusion The PPM was abnormal in both iRBD and RSWA whereas ASR was enhanced in iRBD. We suggest that there are certain similarities and differences in the pathophysiologies of iRBD and RSWA.

Occipital nerve stimulation (ONS) is a specific form of peripheral neuromodulation used in the treatment of chronic pain disorders. A particular field of application is in the therapy of treatment-refractory headaches, especially of chronic migraine. The precise mode of action is unknown. It is presumed that central and peripheral sensitization are reduced in patients with chronic headache. The aim of this study was to examine the effect of ONS on pain-modulatory mechanisms in the trigeminocervical area in patients with chronic migraine. In a balanced repeated measurements design in eight patients with chronic migraine with and without active ONS, we analyzed which effects ONS had on the orbicularis oculi reflex dynamically elicited by corneal air flow. The orbicularis oculi reflex in active ONS (7.38 ± 20.14 eyelid closures/minute) compared to inactive ONS (18.73 ± 14.30 eyelid closures/minute) is significantly reduced (p = 0.021). The results show that under active ONS compared to inactive ONS in patients with chronic migraine, the orbicularis oculi reflex, dynamically triggered by a standardized air flow, is significantly reduced. This suggests that ONS is able to directly counteract the trigeminally mediated central sensitization in chronic migraine and protectively reduce the effects of aversive peripheral stimulation.

Background Neurophysiological markers in dystonia have so far not been sistematically applied in clinical practice due to limited reproducibility of results and low correlations with clinical findings. Exceptions might be represented by the blink reflex (BR), including its recovery cycle (BRRC) and the trigemino-cervical reflex (TCR) which, compared to other neurophysiological methods, have shown more consistent alterations in cervical dystonia (CD). However, a comparison between the two techniques, and their possible correlation with disease symptoms, have not been thoroughly investigated. Objectives To assess the role of BR, BRCC and TCR in the pathophysiology of idiopathic cervical dystonia. Methods Fourteen patients and 14 age-matched healthy controls (HC) were recruited. Neurophysiological outcome measures included latency of R1 and R2 components of the BR, R2 amplitude, BRRC, latency and amplitude of P19/N31 complex of TCR. Clinical and demographic features of patients were also collected, including age at disease onset, disease duration, presence of tremor, sensory trick and pain. The Toronto Western Spasmodic Torticollis Rating Scale was used to characterize dystonia. Results Compared to HC, CD patients showed increased latency of the BR R2 and decreased suppression of the BRRC. They also showed increased latency of the P19 and decreased amplitude of P19/N31 complex of TCR. The latency of P19 component of TCR was positively correlated with disease duration. Conclusions We propose that the increased latency of R2 and P19 observed here might be reflective of brainstem dysfunction, mediated either by local interneuronal excitability changes or by subtle structural damage.

MFS has been reported to recur in 10-12% of patients. There may be a genetic component related to HLA-DR2. Anti-GAD antibodies can be present in MFS along with anti-GQ1b. Common EMG/NCS associations consist of a predominantly axonal, sensory polyneuropathy and absent H reflexes. A 32-year-old female with a history of hypothyroidism presented to our institution twice with symptoms of diplopia, lower extremity weakness and distal paresthesias occurring a year apart. She had ophthalmoplegia, reduced reflexes, and ataxia on exam. CSF showed a borderline elevated protein of 47 and white blood cells

Introduction: This study aimed to investigate the possible excitability changes in the trigeminovascular system based on blink reflex (BR) in patients experiencing migraine and to compare migraine aura attacks and attack-free period. Materials and Methods: A total of 56 patients diagnosed with migraine headache with aura and 20 healthy individuals without migraine were evaluated electrophysiologically using the BR test. The BR test was repeated twice in patients with migraines during attacks with aura and attack-free period. Results: During the migraine attack with aura, R1 latencies were shorter, and R2 latencies were longer than in the interictal period. Likewise, R1 latencies were shorter, and R2 latencies were longer, in the interictal period compared to normal values obtained in the control group. Conclusion: The detected BR abnormalities have been thought to be able to reflect migraine-related dysfunction in the brainstem and trigeminovascular connections. Significance: It has been concluded that the detected BR abnormalities might reflect migraine-related dysfunction in the brainstem and trigeminovascular connections, indicating increased neuronal excitability in migraine.

A literature review was performed on the use of electrodiagnostic (EDX) tests including nerve conduction study, electromyography, exteroceptive reflex, blink reflex, and late response in the evaluation of patients with stiff person syndrome (SPS). A web survey was conducted to report the extent of EDX testing usage in the evaluation of SPS among laboratories accredited by the American Academy of Neuromuscular and Electrodiagnostic Medicine. Coactivation of selected agonist and antagonist muscles was performed in 5 healthy subjects to determine its specificity for SPS. Observation of continuous motor unit activity on electromyography and elicitation of exteroceptive reflexes by electric stimulation are informative in assisting a diagnosis of SPS, but further studies focusing on their sensitivities in diagnosing SPS and specificities in differentiating SPS from other movement disorders are needed. The value of EDX testing in SPS lies in ruling out other neuromuscular disorders.

Objective To characterize the frequency and patterns of optic, trigeminal, and facial nerve involvement by neuroimaging and electrophysiology in IgG4 anti‐neurofascin 155 antibody‐positive (NF155+) chronic inflammatory demyelinating polyneuropathy (CIDP). Methods Thirteen IgG4 NF155+ CIDP patients with mean onset age of 34 years (11 men) were subjected to neurological examination, blink reflex, and visual‐evoked potential (VEP) testing, and axial and/or coronal T2‐weighted head magnetic resonance imaging (MRI). Results Among 13 patients, facial sensory impairment, facial weakness, and apparent visual impairment were observed in three (23.1%), two (15.4%), and two (15.4%) patients, respectively. All 12 patients tested had blink reflex abnormalities: absent and/or delayed R1 in 11 (91.7%), and absent and/or delayed R2 in 10 (83.3%). R1 latencies had strong positive correlations with serum anti‐NF155 antibody levels (r = 0.9, P ≤ 0.0001 on both sides) and distal and F wave latencies of the median and ulnar nerves. Absent and/or prolonged VEPs were observed in 10/13 (76.9%) patients and 17/26 (65.4%) eyes. On MRI, hypertrophy, and high signal intensity of trigeminal nerves were detected in 9/13 (69.2%) and 10/13 (76.9%) patients, respectively, whereas optic nerves were normal in all patients. The intra‐orbital trigeminal nerve width on coronal sections showed a significant positive correlation with disease duration. Interpretation Subclinical demyelination frequently occurs in the optic, trigeminal, and facial nerves in IgG4 NF155+ CIDP, suggesting that both central and peripheral myelin structures of the cranial nerves are involved in this condition, whereas nerve hypertrophy only develops in myelinated peripheral nerve fibers.

Background Bell’s palsy (BP) is a common idiopathic cranial mononeuropathy. The electrophysiological tests are one of supportive tools for localizing the site of facial nerve lesion, and to determine the severity and prognosis of the injury. The aim of this study was to evaluate the role of electrophysiology in the prediction of outcome of patients with Bell’s palsy. This study was carried out on 30 adult patients with Bell’s palsy. All study subjects were assessed clinically by Sunnybrook facial nerve grading score (SBS) and electrophysiologically by electroneurography (ENoG), blink reflex (BR), and electromyography (EMG); first evaluation was within 7th to10th day from onset, the second evaluation was done at 20th day, and the follow-up visit was after 1 month to assess the clinical grading system (SBS). Results At 1 month, 20 patients (66.7%) had good recovery, while 10 patients (33.3%) had poor recovery according to the Sunnybrook scale. Multiple logistic regression analysis showed that the most significant predictive indicator of BP recovery was ENoG value and R1 latency of BR test. The receiver operating characteristic (ROC) curves showed ENoG degeneration index of 74.6%, considered as a critical cutoff value of non-recovery, with the sensitivity 95% and specificity 90%, and the highest specificity was with blink reflex parameters (R1, R2 latency) 100%. Conclusion BR and ENoG were the best predictors of non-recovery of facial function, while EMG findings did not add any prognostic significance. The combination of both tests BR and ENoG, along with clinical findings can be considered a good indication in the acute phase of BP for the possibility to develop palsy residua.

Objective To investigate the prognostic and predictive value of intraoperative blink reflex (BR) monitoring during microvascular decompression (MVD) for hemifacial spasm (HFS). Methods We retrospectively reviewed 41 patients with HFS undergoing MVD with intraoperative BR and lateral spread response (LSR) monitoring. Facial spasm was evaluated for six months postoperatively. Results The BR resolved in 38 patients and persisted in three after MVD. For patients who exhibited BR resolution, 1-day, 1-month, and 6-month follow-ups revealed that 35 (92.1%), 35 (92.1%), and 38 (100%) patients had spasm resolution, respectively. However, of the three patients with persistent BR, one (33.3%), one (33.3%), and zero (0%) patients exhibited spasm resolution at the three corresponding follow-ups. We found a statistically significant difference in spasm resolution between the persistent and resolved BR groups at six months postoperatively. A comparison between intraoperative BR and LSR monitoring revealed that BR was a better predictor of clinical outcomes. Conclusions Intraoperative BR monitoring is a potentially useful tool to help facilitate an adequate decompression and is a reliable prognostic predictor of surgical outcome. Significance This study is the first to document the relationship between intraoperative BR monitoring and surgical outcome in patients with HFS.

Purpose To investigate whether impaired lacrimal pump function is a possible cause of discharge in patients wearing an artificial eye compared with the remaining healthy eye. Methods Consecutive patients wearing unilateral ocular prosthesis for ≥6 months were included in this retrospective study. Excluded were any deformities of eyelids or nasal passage, socket complications such as entropion, ectropion, ptosis, infection, pyogenic granuloma, contracted socket, obstruction of nasolacrimal duct diagnosed with lacrimal irrigation and a difference greater than 2 mm in terms of protrusion between two eyes detected by Hertel exophthalmometry. Patients were asked to score the levels of tearing and mucopurulent discharge between 0 and 5 to assess lacrimal drainage function subjectively. Furthermore, dacryoscintigraphy was performed to assess the functional status of the lacrimal system objectively. Results Included were 32 subjects (12 females, 20 males; aged 32.94 ± 17.62, range 13-78). Mean duration of prosthetic wearing 26.41 ± 21.30 (6-72) months. The mean subjective scores of tearing and mucopurulent discharge were 1.56 ± 1.67 and 1.94 ± 1.63, respectively. The rate of functional stenosis was significantly higher in the anophthalmic socket side as compared to the healthy side (P = 0.002). The rates of a presac, preduct, and intraduct obstruction was notes as n = 9, n = 10, n = 1 and n = 0, n = 6, n = 2 in the anophthalmic side and the companion eye, respectively (P = 0.021). Conclusion Compared to paired healthy eyes, the ocular prosthesis exhibited significantly higher rates of functional lacrimal duct obstruction, especially at the presac level. The alterations in orbital volume and tear film composition in addition to reduced corneal reflex blinking may lead to the failure of lacrimal pump function in artificial eyes.

Electrophysiological studies can provide objective and quantifiable assessments of movement disorders. They are useful in the diagnosis of hyperkinetic movement disorders, particularly tremors and myoclonus. The most commonly used measures are surface electromyography (sEMG), electroencephalography (EEG) and accelerometry. Frequency and coherence analyses of sEMG signals may reveal the nature of tremors and the source of the tremors. The effects of voluntary tapping, ballistic movements and weighting of the limbs can help to distinguish between organic and functional tremors. The presence of Bereitschafts-potentials and beta-band desynchronization recorded by EEG before movement onset provide strong evidence for functional movement disorders. EMG burst durations, distributions and muscle recruitment orders may identify and classify myoclonus to cortical, subcortical or spinal origins and help in the diagnosis of functional myoclonus. Organic and functional cervical dystonia can potentially be distinguished by EMG power spectral analysis. Several reflex circuits, such as the long latency reflex, blink reflex and startle reflex, can be elicited with different types of external stimuli and are useful in the assessment of myoclonus, excessive startle and stiff person syndrome. However, limitations of the tests should be recognized, and the results should be interpreted together with clinical observations.

Studies showed a relatively prolonged blink R1 latency in patients with diabetic distal symmetrical polyneuropathy (DSPN) compared to that without DSPN. We tested the hypothesis that blink R1 latency would provide a diagnostic alternative to nerve conduction studies (NCS) in DSPN and act as a marker of the severity of NCS abnormalities in DSPN.A total of 109 patients with type 2 diabetes underwent blink reflex studies and NCS. We used the composite amplitude scores of nerve conductions (CAS), which consisted of motor (tibial, peroneal and ulnar) and sensory (sural and ulnar) amplitudes for estimating the severity of NCS.Patients with DSPN had longer blink R1, R2, and contralateral R2 latencies (P 0.0001, P = 0.001, and P = 0.031, respectively) and higher CAS (P 0.0001). Area under curve on receiver operating characteristic curve analysis in diagnosing occurrence of DSPN in blink R1 latency was 0.772 (P 0.0001). Multiple linear regression analysis showed that blink R1 latency was independently associated with CAS.Blink R1 latency may be valuable in auxiliary diagnosis and in determining the severity of NCS abnormalities in DSPN.Blink R1 latency can be added as a supplemental marker of severity of NCS in DSPN, especially if the patient's sural amplitudes has a floor effect.

Glabellar tap or reflex (GR) is an old bedside clinical test used in the diagnostics of Parkinson’s disease (PD), but its diagnostic value is unclear. This study examines the diagnostic validity and reliability of GR in PD in relation to brain dopaminergic activity. GR was performed on 161 patients with PD, 47 patients with essential tremor (ET) and 40 healthy controls immediately prior to dopamine transporter (DAT) [123I]FP-CIT SPECT scanning. The binding ratios were investigated with consideration of the GR result (normal/abnormal). In addition, the consistency of the GR was investigated with 89 patients after a mean follow-up of 2.2 years. PD and ET patients had higher GR scores than healthy controls (p p = 0.09). There were no differences in the ratio of abnormal to normal GRs between the PD and ET groups (73% vs. 64% abnormal, respectively, p = 0.13) or in DAT binding between PD patients with abnormal and normal GRs (p > 0.36). Over follow-up, the GR changed from abnormal to normal in 20% of PD patients despite the presence of clinically typical disease. The sensitivity and specificity of GR for differentiating PD from ET were 78.3% and 36.2%, respectively. Although GR has been used by clinicians in the diagnostics of PD, it does not separate PD from ET. It also shows considerable inconsistency over time, and abnormal GR has no relationship with dopamine loss. Its usefulness should be tested for other clinical diagnostic purposes.

Background Superficial eye disorders are one of the most common complications of improper eye care in intensive care units that can lead to corneal ulcers and permanent eye damage. The aim of this study was to determine the effect of the implementation of eye care protocol on the incidence of infection and superficial eye disorders in patients admitted to intensive care units. Methods This study was a cross-over clinical trial that was performed on 32 patients admitted to the intensive care unit with reduced or no blink reflex following loss of consciousness or receiving sedatives. The eye of the test group received eye care according to the protocol and the eye of the control group received the routine care of the ward. The data collection form included demographic and clinical information and the clinical score scale of superficial eye disorders, which were completed in 7 days for both eyes. Data analysis was performed by McNemar and Cochran tests with a 95 % confidence interval. Results In the study of superficial eye disorders, the frequency of dacryorrhea and hyperemia was not significantly different in the second to seventh days in the control and test eyes (P P P = 0.0001). Also, the frequency of corneal opacity was not significantly different in the second and third days (P P P = 0.0001). Conclusions Based on the results, although the implementation of eye care protocol has been able to have a significant effect on reducing ocular complications and problems, routine eye care in the intensive care unit also has clinical effectiveness. Therefore, in order to prevent and completely eliminate eye disorders in the intensive care unit, more evidence and research are needed. Trial registration The trial was retrospectively registered on https://en.irct.ir/trial/43493 on 13 November 2019 (13.11.2019) with registration number [IRCT20140307016870N5].

IntroductionElevated temporal discrimination thresholds (TDT) have been found in cervical dystonia (CD) and unaffected first-degree relatives, indicating autosomal dominant inheritance with reduced penetrance, serving as an endophenotype and being indicative of abnormal inhibitory processing within the brainstem-basal ganglia circuits. The blink reflex R2 recovery cycle (BRRC) is also a measure of excitability of brainstem-basal ganglia circuits, and inconsistent findings are reported in CD. The aim was to investigate TDT and BRRC in CD and evaluate its reliability as an endophenotype.Methods29 patients with isolated cervical dystonia (mean age: 56.1 ± 14.3, female n = 18) and 29 age- and gender-matched healthy controls (mean age: 56.0 ± 14.2, female n = 18) were evaluated using a TDT-paradigm, performed as previously described by testing visual, tactile and visual-tactile temporal discrimination thresholds, and the BRRC, investigated with electrical and air puff stimulation.ResultsMean visual-tactile (p = 0.001) and visual TDTs (p = 0.015) differed between CD and controls; tactile TDTs revealed no group differences (p = 0.232). No between group differences were found for BRRC using either electrical or air puff stimulation (p = 0.117). There was no correlation between the elevation of TDTs and the degree of BRRC-inhibition in CD.ConclusionOur findings support the hypothesis that the TDT is an endophenotype in CD. BRRC testing did not demonstrate disinhibition of brainstem-basal ganglia circuits in CD. In contrast to TDT, the BRRC seems not to represent an endophenotype in cervical dystonia.

Orofacial pain syndromes encompass several clinically defined and classified entities. The focus here is on the role of clinical neurophysiologic and psychophysical tests in the diagnosis, differential diagnosis, and pathophysiological mechanisms of definite trigeminal neuropathic pain and other chronic orofacial pain conditions (excluding headache and temporomandibular disorders). The International Classification of Headache Disorders 2018 classifies these facial pain disorders under the heading Painful cranial neuropathies and other facial pains. In addition to unambiguous painful posttraumatic or postherpetic trigeminal neuropathies, burning mouth syndrome, persistent idiopathic facial and dental pain, and trigeminal neuralgia have also been identified with neurophysiologic and quantitative sensory testing to involve the nervous system. Despite normal clinical examination, these all include clusters of patients with evidence for either peripheral or central nervous system pathology compatible with the subclinical end of a continuum of trigeminal neuropathic pain conditions. Useful tests in the diagnostic process include electroneuromyography with specific needle, neurography techniques for the inferior alveolar and infraorbital nerves, brain stem reflex recordings (blink reflex with stimulation of the supraorbital, infraorbital, mental, and lingual nerves; jaw jerk; masseter silent period), evoked potential recordings, and quantitative sensory testing. Habituation of the blink reflex and evoked potential responses to repeated stimuli evaluate top-down inhibition, and navigated transcranial magnetic stimulation allows the mapping of reorganization within the motor cortex in chronic neuropathic pain. With systematic use of neurophysiologic and quantitative sensory testing, many of the current ambiguities in the diagnosis, classification, and understanding of chronic orofacial syndromes can be clarified for clinical practice and future research.

The aim of the study was to evaluate whether there was a problem in the interneuronal junctions of patients with central sleep apnea using the blink reflex test. Twenty-one (21) patients with central type apneas and/or hipopneas and 21 controls were recruited. The blink reflex test was performed and R1, R2 and contralateral R2 (R3) wave values of both groups were recorded in milliseconds (ms). The results of blink test in patients with central sleep apnea were right side R1 = 9.45 ± 4.35 ms, R2 = 33.35 ± 9.97 ms, R3 = 32.15 ± 10.05 ms and left side R1 = 9.90 ± 3.82 ms, R2 = 31.5 ± 10.5 ms, R3 = 29.00 ± 4.77 ms, (p = 0.49, p = 0.69, p = 0.60, respectively), whereas the results of blink test in controls were right side R1 = 9.25 ± 3.50 ms, R2 = 32.75 ± 11.4 ms, R3 = 32.50 ± 13.12 ms and left side R1 = 7.35 ± 2.6 ms, R2 = 27.75 ± 9.75 ms, R3 = 26.65 ± 14.5 ms. (p = 0.015, p = 0.04, p = 0.035, respectively). All left side (R1, R2, R3) blink reflex of the patients with central sleep apnea was statistically significant prolonged than controls. However, there was no statistically significant difference between women with central sleep apnea and controls regarding all right side (R1, R2, R3) blink reflex. There was statistically significant difference in R1, R2 and R3 waves of left sides blink reflex between two groups. The results may be related to central nervous system damage or/and degeneration in the interneuronal junctions of the brain stem in patients with central sleep apnea syndrome.

Background: The degree of distress caused by the sequelae of peripheral facial nerve palsy usually depends on the severity of synkinesis.Objective: To clarify whether electroneurography (ENoG) can ...

PParry–Romberg syndrome, also known as progressive hemifacial atrophy, is a rare clinical entity of unknown origin with atrophy of skin and subcutaneous fat changes. A classic case of Parry–Romberg syndrome in a woman age 43 is presented. Initial facial changes appeared at the age of 20 years amid complete well-being and in the next 21 years a subcutaneous fat atrophy of the left half face developed, resulting in characteristic deformity changes. No other complaints and manifestations of the disease (epileptic seizures, headache, involvement of the other half of the face and/or extremities) were present at the timeof survey. The analysis of blink reflex, galvanic skin reflex, needle electromyography of the m. orbicularis oris from two sides, computed tomography of the skull bones revealed no pathological changes. The face infrared thermography confirmed asymmetric reduction of temperature on the ipsilateral side. Magnetic resonance scan showed hypotrophy of the left masticatory muscle, the left submandibular salivary gland, thinning of subcutaneous fat layer, changes in the white matter of the brain. Despite the typical clinical features the Parry - Romberg syndrome is a diagnostic challenge known and it is necessary to inform doctors about the latest achievements in the study of etiology, management and prognosis of the disorder.

Purpose: This study analyzes the effect of oxaliplatin treatment on the facial nerve. The facial nerve is the most commonly paralyzed cranial motor nerve because it advances through a long, curved bone canal. Electroneurography and blink reflex are the electrophysiological measurements used for evaluating facial nerve function. Oxaliplatin is a cytotoxic agent used in adjuvant or palliative systemic therapy for colorectal cancer treatment. Methods: This study was performed on 20 individuals who were at least 18 years old at Hacettepe University Ear Nose Throat Department, Audiology and Speech Disorders Unit, and Neurology Division EMG Laboratory as they received oxaliplatin treatment from Hacettepe University Oncology Hospital. Electroneurography and blink-reflex values were recorded and examined. The parameters taken during the second and fourth months were compared for this purpose. Results: This study shows that the prolongation of distal latencies of compound muscle action potential is statistically significant, the amplitudes showed no difference. The ENoG results were analyzed, the prolongation of latency measurements between pre-treatment and the fourth month after treatment were statistically significant. The blink-reflex results showed that comparison with the baseline values, the prolongation of latencies in R1 measurements between pre-treatment, the second month, and the fourth month were significant. Conclusions: The facial nerve is affected asymptomatically by oxaliplatin treatment. During oxaliplatin treatment, the evaluation of facial nerve function could be beneficial for patients by improving their quality of life. Electroneurography and blink-reflex tests can be used in the early evaluations of different medicines to determine their neurotoxicity.

Background The excitability of brainstem interneuronal circuits is partly under control from descending inputs. Since high frequency repetitive transcranial magnetic stimulation (rTMS) modulates cortical output, we hypothesized that it will also modulate brainstem functions. Such modulation may be different in healthy subjects than in subjects with spinal cord injury (SCI), submitted to an altered integration of body afferent inputs. Methods In this randomized, double-blind, sham-controlled trial, we recruited 22 subjects with SCI assigned to either real (n = 11) or sham (n = 11) rTMS and nine healthy subjects, who served as control group, receiving both real (at 20 Hz, with double cone coil over vertex) and sham rTMS separated by at least one week. We recorded the blink reflex (BR) to supraorbital nerve (SON) electrical stimulation and its modification by another conditioning SON, to study the BR excitability recovery (BRER), or a prepulse electrical stimulus to the right index finger, to study the BR inhibition by prepulse (BRIP). Subjects were examined immediately before and after either sham or real rTMS. Results Real but not sham rTMS significantly reduced the area of the BR R2 response in both SCI and healthy subjects. There were no changes in BRER and BRIP. Conclusion rTMS over the vertex modulates brainstem reflexes with no significant differences between SCI and healthy subjects.

Objective Degeneration of nuclei of the brainstem, especially parts of the vagal nuclei complex and the reticular formation, in Parkinson’s disease (PD) may in part be responsible for nonmotor signs like obstipation, cardiac dysfunction and rapid eye movement sleep behavior disorder (RBD). The aim of the study was to establish a new blink reflex (BR) variant involving the vagal nuclei complex and the reticular formation and to investigate BR comprehensively using 3 different afferent routes in PD. Methods In this cross-sectional observational study in 30 PD patients and 30 age and sex matched healthy controls, BR was elicited by stimulation of the auricular branch of the vagus nerve (ABVN) and compared to conventional BR variants evoked by the trigeminal and median nerve. Results BRs could be elicited reliably by stimulation of ABVN in both groups. In none of the three BR variants, latencies or amplitudes differed between PD patients and controls. In PD, BR parameters were not related to cognition or presence of RBD. Conclusion The present study did not provide evidence for malfunctioning of neural circuits subserving BRs elicited by three different afferents in PD. Significance Brainstem circuits mediating these BR variants may be spared from neurodegeneration in PD.

Facial-onset sensory and motor neuronopathy (FOSMN) syndrome represents a rare, slowly progressive, lower motor neuron disease with sensory compromise, involving mainly the face, bulbar region and upper limbs. However, non-motor symptoms and neurogenetic studies have rarely been evaluated in large case series. In the present study, 10 unrelated Brazilian patients with FOSMN syndrome underwent extensive clinical, laboratory, neurophysiological and neurogenetic assessment. Median age at symptom onset was 52.1 years, and men and women were equally affected. Patients presented with hemifacial or bilateral facial paresthesia and weakness, which evolved with dysphagia, dysphonia, and facial and tongue atrophy and, finally, a dropped-head, upper limb weakness and syringomyelia-like sensory disturbances in the upper limbs. All 10 patients showed chronic diffuse neurogenic compromise of bulbar, cervical and thoracic myotomes, and abnormal blink reflex tests. A positive family history of neurodegeneration was identified in six cases, and revealed pathogenic gene variants in three families (involving VCP, TARDBP and CHCHD10). Thus, our case series has revealed new findings regarding FOSMN syndrome: (i) its clinical course is not always benign, with poorer prognoses associated with dropped-head syndrome and early bulbar compromise; (ii) FOSMN syndrome may be part of a complex familial neurodegenerative spectrum; and (iii) a definite genetic basis may be observed in some cases.

Purpose: To observe the inter-rater reliability between the observers for visual acuity, refractive error, corneal reflex, and inter-pupillary distance. Methods: A random subset of 50 students from primary (1st to 4th grade), higher primary (5th to 7th grade) and secondary school (8th to 10th grade), for a total of 150 students were selected by stratified sampling from the prevalence study VARES (Visual acuity, refractive error, and squint) performed among 1752 school children of Udupi district, India in 2013-2014. The Institutional Ethical Committee & District Health Administration board permission was acquired. There were two examiners: Examiner 1 (JT), a qualified optometrist with two years of experience, and Examiner 2 (MT), an optometry student. Examiners 1 and 2 performed a single measurement for visual acuity using the COMPlog computerized logMAR vision chart at 3 metres; Landolt C or Sloan letter optotypes used. Refractive error, corneal reflex, and inter-pupillary distance was measured by Plusoptix Power-refractor twice by Examiner 1 and thrice by Examiner 2; the average of each value was considered for analysis. Data was tested for normality using the Kolmogorov-Smirnov test. Statistical significance was considered p < 0.05. The agreement between the observers for the two procedures was traced by a Bland-Altman plot. Results: The mean age of the students was 10.21 years (± 2.80 SD). The measurement of agreement for visual acuity (COMPlog) between the examiners was tested using Kappa statistics with 0.79 for OD, p < 0.05 and 1.0 for OS, p < 0.05 supported by Wilcoxon sign rank test showing 0.067 for OD and 0.564 for OS between the observers. The Bland-Altman plot also showed good agreement between the observers. Paired t-test for refractive error showed 0.117 (CI: -0.11­–0.10) for OD & 0.067 (CI: -0.005–0.157) for OS, with Bland-Altman plot showing good agreement between the observers. Paired t-test showed 0.323 for corneal reflex and 0.520 for inter-pupillary distance. Conclusion: From the results we report that there is no interobserver variability for visual acuity, refractive error, corneal reflex and inter-pupillary distance in school screening with COMPlog and Plusoptix A09.

Aim: To determine the value of the blink reflex in evaluating trigeminal sensory function during microvascular decompression for trigeminal neuralgia.Methods: The blink reflex (BR) in 103 patients with primary typical trigeminal neuralgia treated by microvascular decompression (MVD) was tested pre- and intraoperatively. The changes in BR were recorded. All patients underwent general anesthesia with intravenous propofol and fentanyl. Surgical efficacy and complications were evaluated after surgery. The relationship between intraoperative changes in the BR and postoperative trigeminal sensory function was analyzed.Results: The BR was elicited in all patients before surgery, and no significant difference was found between the affected side and the contralateral side. In 93 of the 103 cases, the BR was successfully elicited during MVD surgery. Therefore, the recordability of the BR was 90.29%. R1 latency on the affected side and the contralateral side were 11.62 ± 4.96 ms and 11.66 ± 4.37 ms, respectively. During MVD surgery, R1 of the BR disappeared on the affected side in 7 cases and remained in 86 cases. After the operation, 98 of the 103 patients had immediate and complete remission of trigeminal neuralgia symptoms, and 5 cases had partial remission. The 7 patients whose R1 disappeared during the surgery all experienced facial numbness postoperatively. Of the 86 patients whose R1 remained, only 2 patients had postoperative facial numbness. Of the 10 patients whose R1 was not recordable during the operation, one complained of postoperative facial numbness. No patients had complications such as facial paralysis, cerebrospinal fluid leakage, and death.Conclusions: Conclusion: The blink reflex may allow monitoring of trigeminal sensory function during microvascular decompression under general anesthesia.

Objectives Diagnostic criteria of subacute sclerosing panencephalitis (SSPE) include myoclonus, a well-recognized clinical feature. Here, we studied the electrophysiological features of myoclonus with regards to disease staging in SSPE patients. Methods We included 10 patients diagnosed with SSPE between 2010 and 2018, along with 21 healthy subjects. All participants had detailed electrophysiological evaluation including polymyographic analysis, blink reflex after trigeminal stimulation, auditory startle response, startle response after somatosensory stimuli, F-waves, and long-loop reflexes. Clinical findings were retrieved from the medical records. Results Patients were categorized into Gascon stage 2B (n = 5, 50%), 2A (n = 2, 20%), 3B (n = 2, 20%) and 4A (n = 1, 10%) at the time of electrophysiological evaluation. Two patients had cortical myoclonus, four had possible cortico-subcortical myoclonus, and four had brainstem myoclonus. Patients were categorized into Gascon stages 2a and 2b had possible cortico-subcortical myoclonus (85.7%). However, none of the patients with stage 3b or 4a had possible cortico-subcortical subtype but all had the brainstem subtype. Conclusion Association was seen between subtypes of myoclonus and clinical staging in SSPE. This suggests that myoclonus in SSPE may primarily involve the cortex and cortico-subcortical structures such as the thalamus at earlier stages of disease, and then involve more caudal structures as the disease progresses.

Congenital cranial dysinnervation disorders (CCDDs) are a heterogeneous group of neurodevelopmental phenotypes caused by a primary disturbance of innervation due to deficient, absent, or misguided cranial nerves. Although some CCDDs genes are known, several clinical phenotypes and their aetiologies remain to be elucidated. We describe a 12-year-old boy with hypotonia, developmental delay, sensorineural hearing loss, and keratoconjunctivitis due to lack of corneal reflex. He had a long expressionless face, severe oromotor dysfunction, bilateral agenesis/severe hypoplasia of the VIII nerve with marked atresia of the internal auditory canals and cochlear labyrinth malformation. Trio-exome sequencing identified a homozygous loss of function variant in the NEUROG1 gene (NM_006161.2: c.202G > T, p.Glu68*). NEUROG1 is considered a causal candidate for CCDDs based on (i) the previous report of a patient with a homozygous gene deletion and developmental delay, deafness due to absent bilateral VIII nerves, and severe oromotor dysfunction; (ii) a second patient with a homozygous NEUROG1 missense variant and corneal opacity, absent corneal reflex and intellectual disability; and (iii) the knockout mouse model phenotype which highly resembles the disorder observed in humans. Our findings support the growing compelling evidence that loss of NEUROG1 leads to a very distinctive disorder of cranial nerves development. info:eu-repo/semantics/publishedVersion

Neurotrophic keratopathy is characterized by decreased corneal sensitivity, decreased reflex tearing, and poor corneal healing resulting in corneal injury. Without proper sensory innervation, the cornea undergoes continuous epithelial injury, ulceration, infection and eventually results in vision loss. In situations where patients have concomitant facial paralysis, such as after resection of a large vestibular schwannoma, the ocular health is further impaired by paralytic lagophthalmos with decreased eye closure and blink reflex, decreased tearing, and potential lower eyelid malposition. In patients with a dual nerve injury, the ocular surface is in significant danger, as there is increased environmental exposure in conjunction with the inability to sense damage when it occurs. Immediate recognition and care of the eye are critical for maintaining ocular health and preventing irreversible vision loss. The first modern corneal neurotization procedure was described in 2009. The ultimate goal in corneal neurotization is to establish sub-basal plexus regeneration via transferring a healthy nerve to the corneo-limbal region. Corneal neurotization can be achieved either via a direct transfer of healthy nerve (direct approach) or via nerve graft interpositions (indirect approach). This is an emerging concept in the treatment of neurotrophic/exposure keratitis and over the past decade multiple direct and indirect approaches have been described in the attempt to restore corneal sensation and to prevent the devastating outcomes of neurotrophic keratitis. Knowledge of these techniques, their advantages, and disadvantages is required for proper management of patients suffering from neurotrophic keratitis in the setting of facial paralysis.

Study Objectives: This retrospective study investigated prognostic factors and recovery time in patients with Bell’s palsy after different doses and durations of oral glucocorticoid treatments. Subjects and Methods: A total of 396 patients initially diagnosed with Bell’s palsy that had visited the Department of Neurology of Chang Gung Memorial Hospital, Taoyuan, a tertiary referral medical center in Taiwan, between January 2014 and December 2018 were included. Medical records, facial electroneurography (fENoG), and blink reflex (BR) tests were reviewed and analyzed. A favorable outcome was defined as patients who improved to grade ≤ II, and an unfavorable outcome was defined as patients who improved to grade ≥ III in 6 months according to the House–Brackmann (HB) grading system. Results: The rate of favorable outcomes was 89.4% (354 of 396 patients) at the 6-month follow-up. A favorable outcome (HB less than grade II) was associated with a delayed BR (odds ratio, OR, 5.38, 95% CI, 1.82 to 15.90) and fENoG values (the lesion side/the healthy side) over 33% (OR, 6.67, 95% CI, 3.02 to 14.71). The recovery time was significantly shorter for those with a delayed BR than for those with an absent BR and shorter for those with good fENoG values (&gt, 33%) than for those with poor values (≤33%). However, treatment without or with different doses and durations of oral glucocorticoid did not influence the final outcome or recovery time in this study. Conclusions: The fENoG and BR tests were significant and highly valuable examinations for predicting the final outcome. Moreover, age younger than 60 years, a delayed BR, and fENoG values &gt, 33% were associated with shorter recovery times.

Microvascular decompression is the first choice for treating the primary trigeminal neuralgia to provide the most extended duration of pain freedom. However, in microvascular decompression, we found that this kind of operation is only suitable for some patients. It is of great value to objectively judge the function and abnormality of the trigeminal pain conduction pathway in guiding the operation process. This brief report investigates the value of pain evoked potential by electrical stimulation and noceciptive blink reflex in trigeminal neuralgia. We detected the pain evoked potential in 34 patients with trigeminal neuralgia and 48 healthy controls treated by electrical stimulation and blink reflex. We demonstrated no significant differences in the latencies of V1, V2, V3, and R2 of the affected side and the contralateral side in patients with trigeminal neuralgia. The latencies of those four indicators of the affected side in patients with trigeminal neuralgia were notably decreased compared to those on the same side in healthy controls. The receiver operating characteristic curve analysis showed that the area under curve, sensitivity and specificity of the combined diagnosis of latency and amplitude were significantly higher than the single diagnosis. The latency and amplitude of V1 were highly sensitive, while those of V2 was highly specific. Trigeminal neuralgia can be effectively diagnosed by combining pain evoked potential by electrical stimulation and noceciptive blink reflex. The pathogenesis of trigeminal neuralgia should be combined with peripheral pathogenicity and the theory of central pathogenicity.

The aim of the electrophysiological evaluation in facial paralysis is the verification of a peripheral lesion, the determination of the extent of the lesion, and the prognosis of a possible regeneration. For this purpose, the most important methods are nerve conduction studies and the needle electromyography. For special questions, blinking reflex studies can be performed. Together with the clinical examination, the healing process, and taking into consideration an underlying disease, the electrophysiological examination provides a decision support for therapeutic interventions. Therefore it is important to know which information the electrophysiological evaluation can provide and what are its limitations.

Postherpetic neuralgia (PHN) is a painful, long-lasting condition as a consequence of nerve damage resulting from a herpes zoster infection. Although there are many different treatments available to reduce pain duration and severity, PHN is often refractory to them and no single therapy shows an effective cure for all cases of PHN, especially for those involving the ophthalmic branch of the trigeminal nerve. Pulsed radiofrequency (PRF) is a minimally invasive procedure for pain treatment that has been practiced over the past decade. However, its clinical efficacy and safety for treating PHN involving the ophthalmic branch of the trigeminal nerve have not been evaluated. Objective. This study aimed to evaluate the efficacy and safety of PRF for treating PHN involving the ophthalmic branch of the trigeminal ganglion. Study Design. An observational study. Setting. All patients received PRF of the ophthalmic branch of the trigeminal nerve, pain intensity was assessed by a visual analogue scale (VAS), and complications before and after PRF stimulation were noted. Methods. Thirty-two patients with PHN of the ophthalmic branch were treated by PRF of the ophthalmic branch with controlled temperature at 42°C for 8 min. Pain relief, corneal reflex, sleep quality, and satisfaction were assessed for all patients. Results. Thirty out of 32 patients (93.75%) reported significant pain reduction after PRF treatment. Twenty-eight of them (87.5%) were satisfied with their sleep and obtained a pain score lower than 3 following the procedure. Only two patients had a recurrence of the severe burning pain and returned to the hospital for other medical therapies 2 weeks after the PRF procedure. No patient lost the corneal reflex. Limitations. This study is an observational study and a nonprospective trial with a short-term follow-up period. Conclusion. PRF of the trigeminal ganglion of the ophthalmic branch can significantly reduce pain sensation and improve sleep quality and satisfaction for PHN of the ophthalmic branch.

Background The transcanal transpromontorial approach has been introduced to remove vestibular schwannomas. As with other techniques, preservation of the facial nerve (FN) is challenging. This pilot study described FN outcomes of patients preoperatively and postoperatively assessed with electromyography (EMG) and blink reflex (BR). Methods Between September 2017 and December 2018, 10 patients (5 men; 5 women; mean age, 59.8 years; age range, 25–77 years) underwent removal of vestibular schwannoma via the transcanal transpromontorial approach. FN assessment using EMG/BR and clinical evaluation with the House-Brackmann (HB) grading scale was performed preoperatively and 2 months postoperatively. If facial impairment was present postoperatively, further analysis was performed 6 months after surgery. Results All 10 patients had normal FN function on preoperative EMG/BR. After 2 months, 4 patients had normal FN function on EMG/BR, 4 patients showed a slight delay of FN responses, 1 patient had moderate dysfunction, and 1 patient had consistent damage. After 6 months, among the 6 patients with a pathologic neurophysiologic study, consistent EMG/BR improvement was shown, with complete recovery in 3 cases. At the last follow-up, 8 patients had HB grade I, 1 patient had HB grade II, and 1 patient had HB grade III in 1, showing progression toward healing in 9 of 10 cases. Conclusions The transcanal transpromontorial approach is an effective procedure for vestibular schwannoma removal. EMG/BR represents an objective evaluation method to verify FN recovery after surgery and confirms the low impact of this surgical procedure on the FN.

Blink reflex provides an objective assessment of the cranial and central nervous systems. However, the relationships between body mass index, dizziness, and BR have not been explored in patients with type 2 diabetes mellitus (T2DM). Moreover, R2 duration, one of the parameters of the blink reflex, has not been studied to date. In the present study, we aimed to investigate the characteristics and influencing factors of blink reflex in patients with T2DM. We included 45 healthy subjects and 105 hospitalized patients with T2DM. The relationships between these parameters and sex, age, body mass index, duration of T2DM, hemoglobin A1c, distal symmetrical polyneuropathy (DSPN), and dizziness symptoms were analyzed. The results showed that blink reflex latencies (including R1, ipsilateral R2, and contralateral R2 latency) were negatively associated with body mass index but were positively correlated with the duration of T2DM. There were no correlations between blink reflex parameters and sex, age, and hemoglobin A1c. Patients with DSPN had longer blink reflex latencies and shorter R2 durations than those without DSPN. Patients with dizziness had longer latencies (including R1, ipsilateral R2, and contralateral R2 latencies) and shorter R2 durations (including ipsilateral R2 and contralateral R2 durations) than those without dizziness. R2 duration was also a predictive factor for blink reflex abnormality. R2 latency was the most sensitive factor and the optimal predictor of dizziness. These results demonstrate that patients with T2DM with low body mass index, longer duration of T2DM, DSPN, and dizziness-related symptoms had more abnormal blink reflex parameters, indicating more serious injuries to the cranial nerves or the central nervous system.

The aim was to determine differences of blink reflex in clinically definite multiple sclerosis (CDMS) and clinically isolated syndrome (CIS) in patients presented with symptoms and signs of brainstem impairment. The study included 20 patients diagnosed with CDMS, 20 with CIS, and 20 healthy controls. We recorded latencies of early (R1) and late component ipsilaterally (R2) and contralaterally (R2’), and occurrence of irritative component (R3). We analyzed data on sex, age, signs of brainstem impairment and magnetic resonance imaging (MRI) findings for the presence of brainstem demyelinating lesions. There was no statistically significant difference between patient groups according to sex, age, symptoms of brainstem involvement and MRI findings. There was no statistically significant difference in R1 component latencies and R2 latencies on the right side. Latencies of R2 on the left and R2’ on the right were statistically longer in CDMS group. There was no difference in the appearance of R3 component. In conclusion, blink reflex was found to be a very sensitive and useful diagnostic tool in the assessment of brainstem structures, especially because abnormalities are seen not only in CDMS but also in CIS. Slowing of the late component as a sign of dysfunction in the efferent part of the reflex arc is not very specific but is a highly sensitive finding., Cilj je bio ispitati razliku refleksa treptaja u bolesnika s dijagnozom klinički definitivne multiple skleroze (CDMS) i klinički izoliranog sindroma (CIS) koji imaju simptome i znakove oštećenja moždanog debla. Istraživanje je obuhvatilo 20 bolesnika s dijagnozom CDMS, 20 s CIS i 20 zdravih ispitanika kao kontrolna skupina. Bilježili smo latencije rane (R1) i kasne komponente ipsilateralno (R2) i kontralateralno (R2’), kao i pojavu iritativne komponente (R3). Analizirali smo spol, dob, simptome i znakove oštećenja moždanog debla, nalaz magnetske rezonancije (MR) s obzirom na prisustvo demijelinizacijskih lezija u području moždanog debla. Nije utvrđena razlika među skupinama bolesnika s obzirom na spol, dob, prisustvo simptoma oštećenja moždanog debla te nalaz MR. Nije bilo razlike u latencijama komponente R1, kao ni u latencijama R2 na desnoj strani. Latencije komponente R2 na lijevoj strani i R2’ na desnoj strani bile su statistički duže u skupini ispitanika s CDMS. Nije bilo razlike u pojavnosti komponente R3. U zaključku, refleks treptaja je vrlo osjetljiv i koristan dijagnostički alat za procjenu funkcije moždanog debla, pogotovo zbog toga što se abnormalnosti ne vide samo u CDMS, nego i u CIS. Usporenje kasne komponente kao znak disfunkcije eferentnog dijela refleksnog luka, iako nije specifičan nalaz, pokazao se kao vrlo osjetljiv nalaz.

Dystonia is a syndrome characterized by sustained or intermittent muscle contractions causing abnormal, often repetitive, movements, postures, or both. It can be primary or secondary and the patient can present with pure dystonia or dystonia along with other movement disorders such as tremor, myoclonus, parkinsonism, etc. Evaluation of a patient presenting primarily with dystonia includes detailed clinical evaluation, haematologic and neurometabolic work up, imaging and neurophysiological/ electrophysiological studies. This presentation will focus on the electrophysiological evaluation of dystonia, which helps in understanding the pathophysiology and better characterization of the abnormal movement. Compared to tremor and myoclonus, electrophysiological evaluation of muscle spasms and dystonia is difficult. The first step is recording surface EMG from the involved muscles for prolonged periods during abnormal movements (dystonia), rest, and with voluntary contraction. The agonists and antagonists should be included to detect co-contraction, and the unaffected muscles of the same or opposite limbs should also be studied to document overflow dystonia if present. During EMG evaluation, patients are also asked to perform various tasks which can induce dystonia, such as mental arithmetic, finger tapping and opening and closing fist of the unaffected hand, writing (in patients with writer’s cramp). EMG is analyzed to determine: (i) the pattern of EMG activity in agonists and antagonists during dystonia (spasm) (ii) duration of each EMG burst (iii) the regularity of occurrence of EMG bursts, (iv) presence of overflow activity in remote muscles while performing discrete voluntary acts, and (v) the difference in the degree of EMG activity (area of rectified EMG) between epochs with and without muscle spasms. Other investigations which are often useful to characterize dystonia or a dystonic syndrome include: (i) H-reflex recovery curve (ii) mechanically and electrically induced long-latency muscle stretch reflex (iii) reciprocal inhibition between antagonistic muscles (iv) brainstem reflexes such as blink reflex (v) cortical SEP (vi) Bereitschaftpotential (BP) and Contingent negative variation (CNV) and (viii) transcranial magnetic stimulation (TMS). TMS specifically helps to study the excitatory and inhibitory changes in the brain in patients with dystonia and may also be a useful tool to monitor therapeutic interventions.

Objective: Patients with Trigeminal Neuralgia often consults a dentist for relief of their symptoms as the pain seems to be arising from teeth and allied oral structures. Basilar artery Dolichoectasia is an unusual and very rare cause of secondary Trigeminal Neuralgia as it compresses the Trigeminal nerve Root Entry Zone. Case reports: We report three cases of Trigeminal Neuralgia caused by Basilar artery Dolichoectasia compression. The corneal reflex was found absent in all three of the cases along with mild neurological deficits in one case. Multiplanar T1/T2W images through the brain disclosed an aberrant, cirsoid (S-shaped) and torturous Dolichoectasia of basilar artery offending the Trigeminal nerve Root Entry Zone. Discussion: Based on these findings we propose a protocol for general dentist for diagnosis of patients with trigeminal neuralgia and timely exclusion of secondary intracranial causes. Conclusion: General dentists and oral surgeons ought to consider this diagnosis in patients presenting with chronic facial pain especially pain mimicking neuralgia with loss of corneal reflex or other neurosensory deficit on the face along with nighttime pain episodes. Timely and accurate diagnosis and prompt referral to a concerned specialist can have an enormous impact on patient survival rate in such cases. KEYWORDS Basilar artery; Cirsoid dolichoectasia; Corneal reflex; Trigeminal neuralgia.

Introduction Congenital facial weakness (CFW) can result from facial nerve paresis with or without other cranial nerve and systemic involvement, or generalized neuropathic and myopathic disorders. Moebius syndrome is one type of CFW. In this study we explored the utility of electrodiagnostic studies (EDx) in the evaluation of individuals with CFW. Methods Forty-three subjects enrolled prospectively into a dedicated clinical protocol and had EDx evaluations, including blink reflex and facial and peripheral nerve conduction studies, with optional needle electromyography. Results MBS and hereditary congenital facial paresis (HCFP) subjects had low-amplitude cranial nerve 7 responses without other neuropathic or myopathic findings. Carriers of specific pathogenic variants in TUBB3 had, in addition, a generalized sensorimotor axonal polyneuropathy with demyelinating features. Myopathic findings were detected in individuals with Carey-Fineman-Ziter syndrome, myotonic dystrophy, other undefined myopathies, or CFW with arthrogryposis, ophthalmoplegia, and other system involvement. Discussion EDx in CFW subjects can assist in characterizing the underlying pathogenesis, as well as guide diagnosis and genetic counseling.

Purpose: To find out the Centrality of Hirschberg reflex in young emmetropic females and to correlate it with asthenopia. Study Design: Cross-sectional study Place and Duration of Study: Outpatients department of Khalid Eye Hospital, Karachi, from January to December 2019. Methods: Six hundred young emmetropic females 10 to 25 years of age were included. We performed the Hirschberg test and Jackson cross cylinder to assess the astigmatism and observed near point of convergence to find out convergence insufficiency. Results: Out of 600 young emmetropic females only one hundred and ninety had a central Hirschberg corneal reflex along with an astigmatism of 0.25 to 0.50 which was observed in one hundred and sixty five patients. One hundred and twenty five had a receded near point of convergence. Conclusion: Acentrality of Hirschberg can be a cause of asthenopia among young emmetropic females. Key Words: Asthenopia, Astigmatism, Emmetropia, Hirschberg corneal reflex.

Objective To report two cases of cranial multineuritis after severe acute respiratory syndrome caused by coronavirus-2. Methods Patients’ data were obtained from medical records of the clinical chart of dell’Angelo Hospital, Venice, Italy. Results The first patient is a 42-year-old male patient who developed, 10 days after the resolution of coronavirus-2 pneumonia and intensive care unit hospitalization with hyperactive delirium, a cranial multineuritis with asymmetric distribution (bilateral hypoglossus involvement and right Claude Bernard Horner syndrome). No albumin-cytologic dissociation was found in cerebrospinal fluid; severe bilateral denervation was detected in hypoglossus nerve, with normal EMG of other cranial muscles, blink reflex, and cerebral magnetic resonance with gadolinium. He presented a striking improvement after intravenous human immunoglobulin therapy. The second case is a 67-year-old male patient who developed a cranial neuritis (left hypoglossus paresis), with dyslalia and deglutition difficulties. He had cerebrospinal fluid abnormalities (albumin-cytologic dissociation), no involvement of ninth and 10th cranial nerves, diffuse hyporeflexia, and brachial diparesis. Discussion Cranial neuritis is a possible neurological manifestation of coronavirus-2 pneumonia. Etiology is not clear: it is possible a direct injury of the nervous structures by the virus through olfactory nasopharyngeal terminations. However, the presence of albumin-cytological dissociation in one patient, the sparing of the sense of smell, and the response to human immunoglobulin therapy suggests an immune-mediated genesis of the disorder.

Migraine is a complex polygenic disorder of the brain. Specific genes might be responsible for the condition due to the considerable clinical, epidemiological and evolutionary variability and interictal neurophysiological properties. Several studies have found that abnormal processing of a wide range of sensory stimuli is characteristic between attacks of patients that suffer from migraines. These neurophysiological abnormalities were significantly correlated between children that have migraines and their affected parents. Similar electrocortical abnormalities have been observed in relatives apparently free of migraine. This intermediate electrophysiological phenotype was linked to the polymorphism of single genes, such as that of MTHFR and ACE, but was further influenced by several environmental factors. Monogenic dominant forms of familial hemiplegic migraine did not show the same neurophysiological patterns as the most prevalent forms of episodic migraine. Therefore, abnormal information processing can be considered a neurophysiological endophenotypic trait associated with the expression of genetic factors that make an individual vulnerable to the non-monogenic forms of migraine.

AimTo describe ocular manifestations in children with congenital insensitivity to pain with and without anhidrosis (CIPA and CIP).MethodsWe reviewed records of eye examinations of 39 children diagnosed with CIPA or CIP. We collected clinical data, with particular attention to ocular surface findings. Corneal sensitivity was tested by presence of a blink reflex upon touching the cornea. Statistical analysis assessed differences in manifestations between the two conditions, and relationships among corneal sensitivity, presence of corneal opacities and visual acuity (VA).ResultsCIPA was diagnosed in 32 children and CIP in 7. The median follow-up periods were 50 months (CIPA group) and 94 months (CIP group). Corneal opacities were present in 23% of CIPA eyes and in 57% of CIP eyes. A blink reflex was positive in 52% of CIPA eyes and in 33% of CIP eyes. We recorded VA ≥20/25 in 36% of CIPA eyes, whereas all patients with CIP had VA ≤20/30. For the whole cohort, we found a negative correlation between a preserved blink reflex and the presence of corneal opacities, and a positive correlation between a preserved blink reflex and VA ≥20/25.ConclusionChildren with congenital insensitivity to pain are prone to develop corneal scarring. Patients with CIP tend to have more severe ocular surface disease than those with CIPA, probably due to more prevalent loss of corneal sensation. In both groups, a preserved blink reflex correlated with good vision. Affected children should have close follow-up to promptly treat ocular surface disease and prevent vision loss.

We have recently reported on brainstem electrophysiological features of tauopathies, showing that R2 Blink Reflex Recovery Cycle (R2BRRC) could detect Progressive Supranuclear Palsy (PSP) from CorticoBasal syndrome (CBS) [1], as early observed by Valls-Sole and coll. [2]. In this letter, we would like to add some practical indications on the use of R2BRRC also for Multiple System Atrophy (MSA), finding R2BRRC cut-off scores to differentiate atypical parkinsonisms (APs), since the differential diagnosis may result difficult when based on clinical features only.

Wallenberg syndrome, or lateral medullar syndrome, is the clinical presentation of the infarct in the territory of posterior inferior cerebellar artery. Its signs and symptoms include vertigo, nystagmus, diplopia, ipsilateral Horner syndrome, facial ruddiness and dry skin, dysphonia, dysphagia, dysarthria, ipsilateral loss of gag reflex, ipsilateral ataxia, ipsilateral impaired taste, ipsilateral facial pain and paresthesia, decreased ipsilateral blink reflex, contralateral hypoalgesia and thermoanaesthesia in the trunk and limbs; and ipsilateral facial hypoalgesia and thermoanaesthesia. Neuroanatomical knowledge is essential to its comprehension, study and diagnosis, because the classic neurological manifestations are easy to explain and understand if function and localization of affected anatomical structures are known as if the posterior cerebral circulation is.

Objective: To report a case of acute bilateral facial nerve palsy in a 24-year-old woman and to present the differential diagnoses, pathophysiology, management and prognosis Methods: Design: Case study Setting: Tertiary Private Hospital Patient: One (1) Result: A 24-year-old woman with fever, joint pains, cough, chest pain, difficulty ambulating and progressive facial muscle weakness was diagnosed with rheumatic fever. Bilateral facial nerve paralysis was noted, and Electromyography-Nerve Conduction Velocity (EMG-NCV) testing with special facial nerve study revealed abnormal facial nerve and blink reflex studies while EMG-NCV of the upper and lower limbs were normal. Audiometry and MRI of the brain and facial nerve were normal while Schirmer’s Test showed decreased tearing in both eyes. The rheumatic fever resolved within 5 days of antibiotics, while Prednisone and physiotherapy resulted in improvement of facial paralysis from House Brackmann V to House Brackmann II-III over a period of 6 months. Conclusion: Idiopathic facial paralysis or Bell’s Palsy is the most common cause of acute unilateral facial paralysis while bilateral facial nerve paralysis is a rare condition. Patients with facial palsy should undergo appropriate diagnostics to determine the underlying condition and to facilitate prompt management. Keywords: facial paralysis, idiopathic; Bell’s palsy