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Introduction: Sjogren's syndrome (SS) is a systemic autoimmune disease that apart from involving the exocrine glands can affect any organ. Involvement of the peripheral nervous system results in a wide spectrum of neuropathic manifestations.Objective: To evaluate the clinico-electrophysiological patterns as well as pathological characteristics of neuropathy in SS patients presenting to a neuromuscular clinic in a tertiary hospital from South India.Materials and Methods: This is a retrospective study from the Departments of Neurology, Rheumatology, and Pathology from Nizam's Institute of Medical Sciences. Twenty-one patients with the diagnosis of SS and peripheral neuropathy, seen between 2010 and 2016 were analyzed. Clinical records, conventional nerve conduction studies, and lip and nerve biopsy reports were collected.Results: Twenty one patients with SS had associated neuropathy. Female-to-male ratio was 2:1. In 14 (66.7%) patients, neuropathy was the initial manifestation, while in 4 (20%), exocrinopathy preceded neuropathy. The patterns of neuropathy included mononeuropathy multiplex (MNM) in 7 patients (30%), ganglionopathy in 4 (20%), length-dependant trigeminal autonomic neuropathy, and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in 2 (10%), and cranial neuropathy in 1 (10%). Eighteen (86%) were seropositive with either anti Ro/SS-A or anti La/SS-B antibodies. Schirmer's test was positive in 13 (61.9%) patients. Nerve biopsy showed vasculitis in 5 patients and demyelinating and axonopathy in 2 patients each.Conclusions: We conclude that neuropathy is frequently the initial presentation of SS. MNM is the most common pattern followed by ganglionopathy. The pattern of neuropathy helps in arriving at the diagnosis of SS. Serology is a useful initial laboratory test. However,confirmation of SS is not by mere serology. Schirmer's test and lip biopsy are equally essential for the diagnosis, especially in seronegative patients when the clinical index of suspicion is high. [ABSTRACT FROM AUTHOR]

Aim: To study the clinical presentation, radiological findings, and therapy responsiveness of patients with biopsy-proven immunoglobulin G4 (IgG4)-related neurological disease. Methods: The study was conducted between January 2016 and March 2018 from the Department of Neurology and Pathology of Nizam's Institute of Medical Sciences. Patients with neurological symptoms and biopsy suggestive of IgG4-related disease (IgG4-RD) were included. These patients were studied for their demographic pattern and clinical presentation. The presence of serological markers such as vasculitic profile and IgG4 levels was analyzed. Radiological findings were studied in detail. Therapeutic agents used and the response to therapy were assessed. Results: There were six cases with IgG4-related neurological disease which were all hypertrophic pachymeningitis. The age ranged from 35 to 64 (mean = 46) years. The clinical presentation was acute in one, subacute in two, and chronic in three patients. The most common presenting symptom was headache (4), followed by gait and/or urinary disturbances (2), paraparesis (1), and diplopia (1). IgG4 levels were elevated in 50% of them. Pseudotumor-like mass and sinovenous thrombosis, not described previously, were seen in one patient. All the patients were treated with oral or intravenous steroid. Rituximab was given in three patients; azathioprine was the steroid-sparing agent in one patient. Those with acute/subacute onset of presentation had an excellent response to steroids. All the patients with a chronic duration of their symptoms received empirical anti-tuberculous therapy before a definitive diagnosis of Ig G4-RD was made. Conclusions: The characterization of patients with IgG4-related neurological disease based on the understanding of the clinical spectrum increases the confidence in the clinician to resort to early immunosuppression, thereby having prognostic implications. [ABSTRACT FROM AUTHOR]

This study described the clinical and paraclinical features of south Indian patients with longitudinally extensive transverse myelitis (LETM) and contrasted the findings betweenaquaporin-4 positive versus negative patients. The subjects were recruited between2010 and 2013.The distinctive features among71 LETM patients were compared and it was observed that 56% of the total subjects were found to be AQP4-Ab positive. The ratio of female tomale was found to be higher in the AQP4-Ab positive group. Magnetic resonance imaging showed holocord involvement more commonly in AQP4- Abnegative than positive group. The presence of hypointense lesions did not correlate with severity. The main distinctive features between AQP4-Abpositive and negative cases include older onset age, higher proportion of female, low frequency of conus involvement and higher prevalence of coexisting autoimmune disorders in AQP4-Ab positive cases. Therewas no difference in attack severity, onset of optic neuritis, and spasms between the two groups. Our results suggest that the clinical and spinal cord neuro-imaging information can aid in distinguishing between the positive and negative group of patients with LETM. The early detection of AQP4-Ab positive status predicts the recurrence of LETM or occurrence of optic neuritis duringthe study period. [ABSTRACT FROM AUTHOR]

Background: The diagnostic accuracy of high-resolution ultrasonography (HRUS) in comparison to electro-diagnostic testing (EDX) in carpal tunnel syndrome (CTS) is debatable. Objective: The aim of this study was to compare the diagnostic accuracy of HRUS with EDX in patients with various grades of CTS and CTS associated with peripheral neuropathy (CTS + PNP). Materials and Methods: A prospective cohort of 57 patients with possible CTS was studied along with matched controls. The cross-sectional area (CSA) of the median nerve at the inlet of carpal tunnel was assessed by a sonologist blinded to the clinical and EDX data. Palm wrist distal sensory latency difference (PWDSLD), second lumbrical-interosseus distal motor latency difference (2LIDMLD) and CSA were compared in patients with different grades of severity of CTS and CTS + PNP. Results: Total 92 hands of 57 patients met the clinical criteria for CTS. Mean CSA at the inlet of carpal tunnel was 0.11 ± 0.0275 cm². It had the sensitivity, specificity, positive predictive value and negative predictive values of 76.43%, 72.72%, 89.47% and 68%, respectively (P < 0.0001). Overall, HRUS had good correlation with PWDSLD and 2LIDMLD electro-diagnostic studies in all grades of CTS and CTS + PNP. Conclusion: HRUS can be used as a complementary screening tool to EDX. However, EDX has been found to be more sensitive and specific in mild CTS. [ABSTRACT FROM AUTHOR]

A letter to the editor about the case report of an 18-year-old medical student with intraneural capillary hemangioma is presented.

Background : Epilepsy with myoclonic absences (EMAs) is a distinct form of childhood epilepsy characterized by a peculiar seizure type that identifies this condition. Purpose : To describe the clinical, electroencephalographic features, treatment strategies and outcome in this first case series of two siblings with normal intelligence presenting with EMAs. Materials and methods : Both siblings underwent video-polygraphic investigations (simultaneous recording of electroencephalogram [EEG] and electromyogram [EMG] from deltoids), high-resolution magnetic resonance imaging (MRI), karyotyping, neuropsychological evaluation and language assessment. Results : Both the children had a mean age of onset of prototype seizures by 3.5 years. Myoclonic absences (MAs) were characterized by rhythmic, bilateral, synchronous, symmetric 3-Hz spike–wave discharges, associated with EMG myoclonic bursts at 3 Hz, superimposed on a progressively increasing tonic muscle contraction. The interictal EEG showed a normal background activity with bursts of generalized spike and waves (SWs) as well as rare focal SWs independently over bilateral temporal and frontal regions. Increase in the seizure frequency from 5 to 100/day was observed due to use of carbamazepine and phenobarbitone which decreased with its withdrawal and introduction of valproate. Though lamotrigine was given as an add on to valproate, it did not benefit them and was therefore replaced by topiramate at 3.5 mg/kg/day which has maintained them on remission at one year follow up. Conclusions : Recognition of this ictal pattern allows identification and differentiation of EMAs from other seizure types. Idiopathic and symptomatic EMAs need to be differentiated from childhood absence epilepsy with myoclonia. MAs are worsened by drugs like carbamazepine while valproate either alone or in combination with topiramate (preferred to lamotrigine) gives excellent outcome. [ABSTRACT FROM AUTHOR]

Background: Subacute sclerosing panencephalitis (SSPE) is a delayed and fatal manifestation of measles infection. Fulminant SSPE is a rare presentation in which the disease progresses to death over a period of 6 months. The clinical features are atypical and can be misleading., Case Report: We report herein a teenage boy who presented with acute-onset gait ataxia followed by right hemiparesis that evolved over 1 month, with left-hemispheric, delta-range slowing on the electroencephalogram (EEG). Magnetic resonance imaging disclosed multiple white-matter hyperintensities, suggesting a diagnosis of acute disseminated encephalomyelitis. He received intravenous steroids, and within 4 days of hospital admission he developed unilateral slow myoclonic jerks. Repeat EEG revealed Rademecker complexes, pathognomonic of SSPE, and an elevated titer of IgG antimeasles antibodies was detected in his cerebrospinal fluid. The disease progressed rapidly and the patient succumbed within 15 days of hospitalization. The diagnosis of SSPE was confirmed by autopsy., Conclusions: This case illustrates the difficulty of recognizing fulminant SSPE when it manifests with asymmetric clinical and EEG abnormalities.