Search

Objectives: To characterize the late cranial neuropathy among 10-year survivors of head and neck cancer treatment., Materials and Methods: We retrospectively evaluated patients treated with curative-intent radiation for HNC between 1990 and 2005 at a single institution with systematic multidisciplinary follow-up ≥ 10 years. New findings of CNP were considered radiation-induced when examination, imaging and/or biopsy did not demonstrate a structural or malignant cause. Cox proportional hazards modeling was used for univariable analysis (UVA) and multivariable analysis (MVA) for time to CNP after completion of radiation., Results: We identified 112 patients with no evidence of disease and follow-up ≥ 10 years (median 12.2). Sixteen (14%) patients developed at least one CNP. The median time to CNP was 7.7 years (range 0.6-10.6 years). Most common was CN XII deficit in eight patients (7%), followed by CN X deficit in seven patients (6%). Others included CN V deficit in three, and CN XI deficit in two. Eight of the thirteen patients with a CN X and/or CN XII deficit required a permanent gastrostomy tube. On UVA, site of primary disease, post-radiation neck dissection, chemotherapy, and radiation dose were significantly associated with increased risk of CNP., Conclusion: Iatrogenic CNP may develop years after head and neck cancer treatment and often leads to swallowing dysfunction. Long-term follow up is essential for these patients receiving head and neck radiation., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

Background: Hypoglossal nerve injury may result in swallowing and speech problems. To reduce this morbidity and allow the performance of the hypoglossal-facial nerve anastomosis bilaterally, a technique that includes partial splitting of the hypoglossal nerve and skeletonization of the facial nerve within the mastoid process has been applied. The aim of this study is to present clinical results regarding the facial and hypoglossal nerves after the procedure., Methods: Prospectively collected data from 56 consecutive patients who underwent hemihypoglossal-facial nerve anastomosis (HHFA) were analyzed. The outcome was correlated with epidemiologic data, initial disease, the presence of neurofibromatosis type 2, previous radiosurgery, and the time between nerve injury and reconstructive surgery., Results: Forty-eight (84%) patients achieved satisfactory outcomes; 8 of them (14%) showed some improvement, and in 1 patient (2%) there was no improvement during long-term observation. The result at follow-up was not related to the time interval between the 2 procedures. However, recovery times for facial tonicity were statistically significantly longer if the procedure was performed after 12 months (P = 0.044). There was no statistically significant association between patient age (P = 0.96) or sex (P = 0.13) and facial nerve function. HHFA resulted in no or minimal tongue atrophy without deviation in 53 patients (93%), and the remainder had mild hemiatrophy with tongue deviation <30 degrees., Conclusions: HHFA is an effective technique for facial nerve reanimation with acceptable morbidity related to tongue function. Patients with a longer duration of facial palsy still have a good chance for restoration of facial movement but require longer recovery periods., (Copyright © 2018 Elsevier Inc. All rights reserved.)

The hypoglossal nucleus was recently identified as a key brain region in which the presence of TDP-43 pathology could accurately discriminate TDP-43 proteinopathy cases with clinical amyotrophic lateral sclerosis (ALS). The objective of the present study was to assess the hypoglossal nucleus in behavioral variant frontotemporal dementia (bvFTD), and determine whether TDP-43 in this region is associated with clinical ALS. Twenty-nine cases with neuropathological FTLD-TDP and clinical bvFTD that had not been previously assessed for hypoglossal TDP-43 pathology were included in this study. Of these 29 cases, 41% (n=12) had a dual diagnosis of bvFTD-ALS at presentation, all 100% (n=12) of which demonstrated hypoglossal TDP-43 pathology. Of the 59% (n=17) cohort that presented with pure bvFTD, 35% (n=6) were identified with hypoglossal TDP-43 pathology. Review of the case files of all pure bvFTD cases revealed evidence of possible or probable ALS in 5 of the 6 hypoglossal-positive cases (83%) towards the end of disease, and this was absent from all cases without such pathology. In conclusion, the present study validates grading the presence of TDP-43 in the hypoglossal nucleus for the pathological identification of bvFTD cases with clinical ALS, and extends this to include the identification of cases with possible ALS at end-stage., (Crown Copyright © 2016. Published by Elsevier B.V. All rights reserved.)

Parkinson's disease (PD) is a common neurodegenerative disorder that is often associated with weak tongue motility. However, the link between the degenerated dopaminergic neurons in the substantia nigra (SN) and lingual dysfunction remains unclear. In the present study, we investigated the localization of dopamine receptor 1 (D1) and dopamine receptor 2 (D2) and alternations in their expression in cholinergic motoneurons of the hypoglossal nucleus (HN) using double-label immunofluorescence, Western blotting and semi-quantitative reverse transcription and polymerase chain reaction (SqRT-PCR) in rats that received microinjections of 6-hydroxydopamine bilaterally into the SN (6-OHDA rats). The results revealed that a large population of choline acetyltransferase immunoreactive (ChAT-IR) neurons was distributed throughout HN and that almost all of the ChAT-IR motoneurons were also D1-IR and D2-IR. Several tyrosine hydroxylase (TH)-IR profiles were observed in a nonuniform pattern near the ChAT-IR, D1-IR or D2-IR somas, suggesting potent dopaminergic innervation. In the 6-OHDA rats, TH immunoreactivity in the SN was significantly decreased, but food residue was increased and treadmill occupancy time was shortened. In the HN, protein expression of TH and D2 was increased, whereas that of ChAT and D1 was decreased. A similar pattern was observed in mRNA levels. The present study suggests that dopamine may modulate the activity of cholinergic neurons via binding with D1 and D2 in the HN. Changes in the expression of ChAT, TH, D1 and D2 in the HN of 6-OHDA rats might be associated with the impaired tongue motility in PD. These findings should be further investigated., (Copyright © 2014 Elsevier Inc. All rights reserved.)

The aim of this retrospective study is to assess the rate at which glomus jugulare tumors invade the hypoglossal canal (HC) and to correlate computed tomography (CT) and magnetic resonance imaging (MRI) findings with the clinical evidence of cranial nerve (CN) XII dysfunction. CT and MRI imaging modalities of 31 patients were blindly reviewed by an attending neuroradiologist. Imaging studies identified involvement in 22 tumors (22/31, 71.0%). Thirteen of 22 patients (59.1%) had clinically evident CN XII symptoms. Accuracy rate was 76.7% (23/30) for MRI and 78.6% (11/14) for CT. MRI showed 100% sensitivity but had only 59% specificity and the specificity for CT was 66.7%. When radiologists elucidate HC involvement, it may alter the surgical approach and may lead to more focused/accurate clinical evaluation of tongue function., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Objective: Posteroinferior cerebellar artery (PICA) aneurysms are uncommon and have not been well investigated previously. We report our series of 29 ruptured PICA aneurysms with surgical treatment along with the description of the surgical anatomy of the PICA to the lower cranial nerves in cadaveric specimen., Methods: All patients with ruptured PICA aneurysms who were surgically treated at the First Affiliated Hospital of Soochow University during the period from January 1995 to December 2008 were reviewed retrospectively. Data relating to clinical, radiological, and intraoperative findings were analyzed. Forty formalin-fixed cerebellar hemispheres provided the material for the study of describing the detailed surgical anatomic relationship of the PICA to the lower cranial nerves., Results: In our series, ruptured PICA aneurysms reached an incidence of 2.35% of all ruptured intracranial aneurysms. There were 13 aneurysms (44.8%) located in the proximal segment, and 16 (55.2%) located in the distal segment. Of these, 89.7% were saccular, 6.9% fusiform, and 3.4% dissecting aneurysms. Usually, the surgical outcome was influenced by Poor admission grade, the presence of obstructive hydrocephalus and associated distal AVM. In cadaveric specimen, 17.5% of PICAs passed between the glossopharyngeal and vagus nerves, 7.5% between the vagus and accessory nerves, and 62.5% through the rootlets of the accessory nerve., Conclusion: This report summarizes the presentation and outcome of a large series of 29 patients with ruptured PICA aneurysms, and we conclude that ruptured PICA with surgical treatment usually gets well recovered. The study does, however, also demonstrate that the anatomic relationship of the PICA and lower cranial nerves is somehow variable and irregular. Recognition of the findings in cadaveric dissection is essential in treating lesions of this region., (Copyright © 2011 Elsevier B.V. All rights reserved.)

Background: The lower cranial nerves must be identified to avoid iatrogenic injury during skull base and high cervical approaches. Prompt recognition of these structures using basic landmarks could reduce surgical time and morbidity., Methods: The anterior triangle of the neck was dissected in 30 cadaveric head sides. The most superficial segments of the glossopharyngeal, vagus and its superior laryngeal nerves, accessory, and hypoglossal nerves were exposed and designated into smaller anatomic triangles. The midpoint of each nerve segment inside the triangles was correlated to the angle of the mandible (AM), mastoid tip (MT), and bifurcation of the common carotid artery., Results: A triangle bounded by the styloglossus muscle, external carotid artery, and facial artery housed the glossopharyngeal nerve. This nerve segment was 0.06 ± 0.71 cm posterior to the AM and 2.50 ± 0.59 cm inferior to the MT. The vagus nerve ran inside the carotid sheath posterior to internal carotid artery and common carotid artery bifurcation in 48.3% of specimens. A triangle formed by the posterior belly of digastric muscle, sternocleidomastoid muscle, and internal jugular vein housed the accessory nerve, 1.90 ± 0.60 cm posterior to the AM and 2.30 ± 0.57 cm inferior to the MT. A triangle outlined by the posterior belly of digastric muscle, internal jugular vein, and common facial vein housed the hypoglossal nerve, which was 0.82 ± 0.84 cm posterior to the AM and 3.64 ± 0.70 cm inferior to the MT., Conclusions: Comprehensible landmarks can be defined to help expose the lower cranial nerves to avoid injury to this complex region., (Copyright © 2010 Elsevier Inc. All rights reserved.)

A neurovascular conflict (NC) consists of a pathological contact between a vessel, generally an artery, and the root entry zone of a cranial nerve close to the brainstem. Even if NC of the V, VII and IX cranial nerve have been rarely described, to the best of our knowledge there is no report about the XII cranial nerve NC in the paediatric age. A three-year-old girl presented with right-sided tongue atrophy and fasciculation, of one-year-duration, consistent with a peripheral lesion of the right XII cranial nerve. Brain MRI and MRA documented a marked tortuosity of the vertebrobasilar arteries compressing the brainstem at the emergency of the XII cranial nerve, while the CT disclosed a concomitant osseous malformation of the cranio-cervical junction. The differential diagnosis of a peripheral unilateral cranial nerve palsy should include, even if rare in children, a neurovascular conflict. In this case a complete neuroimaging study is indicated.

In this study, we examined the expression of mRNAs for Regenerating gene (Reg)/pancreatitis-associated protein (PAP) family members following hypoglossal nerve injury in rats. In addition to four rat family members (RegI, Reg-2/PAP I, PAP II, and PAP III) that had been identified, we newly cloned and sequenced a type-IV Reg gene in rats. Among these five family members, the expression of Reg-2/PAP I mRNA was predominantly enhanced in injured motor neurons after axotomy. Furthermore, a marked induction of PAP III mRNA was observed in the distal part of the injured nerve. A polyclonal antibody was raised against PAP III, and a Western blotting analysis using this antibody confirmed an increased level of PAP III protein in the injured nerve. These results suggest that Reg family members would be new mediators among injured neurons and glial cells, and may play pivotal roles during nerve regeneration.

A rare case of cellular schwannoma of the hypoglossal nerve, with intraspinal extension, presenting without any recognisable impairment of the function of the hypoglossal nerve is presented.

Fracture of the occipital condyle is a rare injury that can be easily overlooked. Palsies of lower cranial nerves can be the only symptom of the fracture. We report a patient with isolated post-traumatic hypoglossal nerve palsy who developed hypoglossal nerve palsy within 2 months after a car accident, indicating that the acute trauma itself did not damage the hypoglossal nerve. Most likely the palsy is caused by pressure to the nerve prior to the entry or within the hypoglossal canal. Since, in the present case, the fracture was stable and the patient showed only moderate neurological deficits, the operation was deferred.

Chromatolytic motor neurons (cMN) in Werdnig-Hoffmann disease (WHD) were investigated in both spinal anterior horns and hypoglossal nuclei with both immuno- and lectin-histochemistry in six cases (3-9 months; two female and four male) of clinically typical WHD. Most characteristic findings from lectin-histochemistry were central accumulation of N-linked glycopeptides and marked general paucity of O-linked glycopeptides in cMN. Phosphorylated intermediate filaments, developmentally regulated cytoskeletons and cell adhesion molecules were abundant at the periphery of cMN, as visualized with immunohistochemistry. Both N-linked and O-linked glycoproteins were reciprocally absent or scarce at the peripheral zone of cMN. This intriguing phenomenon provided the basis for postulating the pathogenesis of WHD. The reciprocal ('yen-yang') dissociation of phosphorylation and glycosylation of neurofilament proteins was only seen with phosphorylated neurofilaments and seen only in cMN, not in the control or surrounding unaffected motoneurons. The central accumulation of N-linked glycopeptides was in contrast with peripheral absence of O-GlcNAc-linked glycopeptides which would normally be expected to colocalize with phosphorylated neurofilaments. Both O-glycosylation and phosphorylation are considered essential for assembly and network of neurofilaments. Aberrant O-glycosylation and dissociation of O-glycosylation/phosphorylation would not only cause a defect in neurofilament assembly but also neuron-glia adhesion (via a molecule such as Ng-CAM), causing a failure of lower motoneurons to synapse homophilically with the upper motoneurons and also a failure to adhere heterophilically to glia, resulting in the histopathologic tetrad ((i) central chromatolysis, (ii) empty-cell beds, (iii) migratory motoneurons and (iv) glial bundles of spinal roots) typical of WHD.

Peripheral nerve injury and neuronal degeneration resulting from toxic ricin induce activation of the classical pathway of complement close to the injured motorneuron perikarya or sensory terminals. In contrast, degeneration of central myelinated fibers is not accompanied by complement expression. The main source of complement in peripheral nerve injury and toxic ricin degeneration appears to be microglia. Brain contusion is associated with complement activation. Some of the complement in this situation may derive from plasma, because the blood-brain barrier is disrupted. Clusterin expression is increased in astrocytes along with their activation in the vicinity of lesioned neurons. In addition, axotomized motorneurons show a marked clusterin upregulation. A relationship between clusterin and cell death is suggested by the prominent aggregation of clusterin in neuronal perikarya destroyed by the effects of toxic ricin, as well as by the neosynthesis of clusterin in apparently degenerating nonneuronal cells, presumed to be oligodendrocytes. Our findings indicate that the expression of complement and clusterin are prominent features of neural degeneration and regeneration, as it is in Alzheimer's disease brains as well. The nerve injury conditions described, therefore, offer attractive experimental models to elucidate the roles of these molecular components in neurodegenerative disorders, thereby providing useful insights into potentially new therapeutic approaches in these conditions.

Background: Finnish-type familial amyloidosis (FAP-IV) is an autosomal, dominantly inherited disorder characterized by progressive polyneuropathy and lattice corneal dystrophy type II. The vast majority of families with this disorder originated from Finland. Only two families, in neighboring districts, have been reported in Japan previously., Methods: The authors report two additional Japanese patients with FAF-IV. The proband, a 70-year-old man, had decreased perspiration and abnormal facial muscle movement. Results of neurologic examination showed bilateral facial and hypoglossal nerve palsies, and an autonomic disturbance, including orthostatic hypotension and dysfunction of perspiration. Histochemical, immunohistological, and DNA studies confirmed the diagnosis of FAP-IV., Results: Results of ophthalmologic examination showed asymptomatic lattice corneal dystrophy of both eyes, but the appearance of the cornea was different from that described in the patients from Finland. Lattice lines in the authors' patient were very fine, short, and glassy and could be observed with indirect retroillumination, but might be missed with direct illumination by the slit-lamp microscope. The proband's younger half-sister, a 68-year-old woman, showed clinical findings and laboratory data similar to those of the proband., Conclusion: The authors report two Japanese patients with lattice corneal dystrophy type II related to FAP-IV. This is the third Japanese family with this disorder, and there is no familial relationship to the two previously reported families in Japan.

The authors report a case of a cervical zoster (C2 - C4) with unilateral involvement of the IXth, Xth, XIth and XIIth cranial nerves. Angiography failed to opacify the ascending pharyngeal artery on the same side, presumably because of a thrombosis secondary to the zoster infection. As the ascending pharyngeal artery is known to supply the last four cranial nerves, this study should be seen as a further example of the varied cranial nerve involvement which may arise on a vascular basis.

A case of Pena-Shokeir I syndrome in a female neonate is reported. The baby died of respiratory insufficiency shortly after birth. Postmortem examination revealed marked pulmonary hypoplasia and microscopic abnormalities of the hypoglossal nucleus and anterior horn cells in spinal cord. A comparative study with respect to hypoglossal nucleus disclosed the possibility of hypoplasia of the hypoglossal nucleus as part of this syndrome. This finding led us to propose that hypoplasia of the nucleus is one of the causes of polyhydramnios, based on the lack of swallowing activity. Moreover, ectopic cross-striated muscle fibers and an increase of muscle spindles were noted in tongue. The findings, particularly the neuropathologic one, provide further insight into the pathogenesis of the syndrome.

The occurrence of neurofibrillary tangles in both the cerebral cortex and brain stem is typically seen in the Guam type of amyotrophic lateral sclerosis, but is exceedingly rare in the classical form of the disease. Only 3 cases of sporadic amyotrophic lateral sclerosis with such histopathologic features have so far been reported, all in the United States. A 49-year-old Brazilian woman had an 18-month history of amyotrophic lateral sclerosis involving predominantly the left-sided extremities with prominent bulbar signs. Autopysi disclosed moderate to severe loss of motor neurones in the hypoglossal nuclei and anterior spinal horns, absence of pyramidal tract demyelination, depigmentation of the substantia nigra and numerous neurofibrillary tangles in the hypothalamic region, parahippocampal gyrus, reticular substance of the mesencephalon and pons and in some brain stem nuclei. The topographical distribution of these changes was closely similar to that of Guamanian amyotrophic lateral sclerosis.