Cases reported "Cranial Nerve Diseases"

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11/714. Surgical findings in idiopathic trigeminal neuropathy mimicking a trigeminal neurinoma.

    Idiopathic trigeminal neuropathy is a benign disorder where the main clinical feature is facial numbness limited to the territory of one or more divisions of the trigeminal nerve, persisting for a few weeks to several years and in which no underlying disease can be identified. The case of a 37-year-old man with a brief history of sensory and motor trigeminal symptoms who showed magnetic resonance imaging (MRI) findings consistent with a small trigeminal neurinoma is reported. The patient was operated on but no tumour could be found during surgery and a biopsy was taken from the portio major. Pathological diagnosis was chronic non granulomatous inflammatory reaction with areas of coagulation necrosis. Control MRI showed complete resolution of the trigeminal abnormalities. It is concluded that in patients with MRI findings suggestive of a small trigeminal schwannoma, benign idiopathic trigeminal neuropathy should also be considered in the differential diagnosis. A conservative approach with sequential MRI studies may avoid an unnecessary surgical exploration. ( info)

12/714. Benign triton tumor of the trigeminal nerve.

    Benign triton tumor (neuromuscular hamartoma) is a rare tumor of the peripheral nerves, comprising mature neural and striated muscle elements. We report the third case, according to the literature, of a benign triton tumor affecting the Vth cranial nerve in a 4-year-old male child. The tumor was seen to involve the mandibular division of the trigeminal nerve in the infratemporal fossa and extend intracranially by eroding the base of middle cranial fossa. The mass was totally excised. In the tumor, bundles of axons intermingling with fascicles of relatively mature skeletal muscle were found. This type of hamartomatous lesions of neuroectodermal-mesenchymal origin could have arisen as the result of incorporation of mesenchymal tissue into nerve sheaths during embryogenesis or aberrant differentiation of neuroectodermal component into mesenchymal elements. ( info)

13/714. Bilateral recurrent nerve paralysis associated with multinodular substernal goiter: a case report.

    Substernal goiter is an infrequent occurrence and is found in two to five per cent of all patients undergoing thyroid surgery. These lesions are well known to cause respiratory symptoms and alterations in phonation due to direct compression of airway structures. Infrequently, unilateral recurrent nerve palsy has been reported in patients with substernal goiter. We report a case of bilateral recurrent nerve palsy associated with multinodular substernal goiter in an 89-year-old female who presented in respiratory distress. ( info)

14/714. herpes zoster of the trigeminal nerve third branch: a case report and review of the literature.

    literature review AND CASE REPORT: A literature review of herpes zoster of the trigeminal nerve is presented. Included are differential diagnosis and treatment modalities that will enable the dental practitioner to identify and manage this disease. A case report is provided to amplify this timely information. ( info)

15/714. Bilateral trochlear nerve palsy associated with cryptococcal meningitis in human immunodeficiency virus infection.

    This is the report of a case of bilateral trochlear nerve palsy secondary to cryptococcal meningitis in a 34-year-old woman with acquired immune deficiency syndrome. Based on clinical and neuroradiologic findings, it is concluded that in the present case, a postinflammatory shrinking of the arachnoid has stretched the fourth cranial nerves at their point of emergence from the dorsal surface of the brainstem. ( info)

16/714. abducens nerve palsy complicating pregnancy: a case report.

    We report a case presented at 38 weeks gestation with abducens nerve palsy. No specific pathology was found. After reviewing all the previously reported cases, hypertension is found to be a common factor in all cases presenting in late pregnancy. The clinical course is benign and all resolved after delivery. ( info)

17/714. Neuromyotonia of the abducens nerve after hypophysectomy and radiation.

    The clinical signs of the rarely encountered ocular neuromyotonia consist of transient involuntary tonic contraction and delayed relaxation of single or multiple extraocular muscles, resulting in episodic diplopia. With a mean time delay of 3.5 years, this motility disorder frequently follows tumor excision or adjuvant radiation near the skull base. Ocular neuromyotonia may reflect inappropriate discharge from oculomotor neurons with unstable cell membranes because of segmental demyelinization by tumor compression and radiation-induced microangiopathy. In the present paper, the authors present the case of a 53-year-old patient with a history of transsphenoidal hypophysectomy and adjuvant radiotherapy, who underwent strabismus surgery for abducens palsy. ( info)

18/714. Ocular amyloidosis and secondary glaucoma.

    OBJECTIVE: To report the clinical and histopathologic findings in two cases of secondary glaucoma associated with amyloidosis. DESIGN: Two case reports. methods: Retrospective review of clinical findings, course, and treatment of the two patients. The histopathologic findings from available biopsy material were also reviewed. MAIN OUTCOME MEASURES: intraocular pressure (IOP), visual field changes, and surgical outcome. RESULTS: The first case describes a 76-year-old woman with orbital amyloidosis who developed gradual unilateral elevation of IOP that was poorly responsive to medical therapy and underwent filtration surgery. Episcleral venous pressure was elevated on the affected side, and histopathologic analysis of the conjunctival tissue confirmed perivascular amyloid deposits, further suggesting raised episcleral venous pressure to be a possible mechanism of glaucoma. The second case describes a 47-year-old white woman with familial amyloid neuropathy with a transthyretin cys-114 mutation. The association of glaucoma with this mutation has not been described previously. Persisting elevation of IOP in one eye was initially responsive to topical antiglaucoma medications but eventually required filtration surgery. Amyloid particles were found in the aqueous and on the lens surface. Histopathologic analysis of the aqueous and sclerectomy specimens demonstrated amyloid, suggesting outflow obstruction as a possible mechanism of glaucoma. Conjunctival buttonholing complicated filtration surgery in both cases, and the leaks eventually resolved with good control of IOP. CONCLUSIONS: Amyloid associated with glaucoma may involve different pathophysiologic mechanisms. The elevated IOP may not respond well to medical therapy. Cautious surgical manipulation of the conjunctiva is warranted in these cases. ( info)

19/714. Peripheral-type primitive neuroectodermal tumor arising in the tentorium. Case report.

    The authors report the case of a peripheral primitive neuroectodermal tumor (PNET) arising in the tentorium in a 5-year-old boy who presented with frequent vomiting and mild palsy of the left abducent nerve. Following complete surgical excision of the tumor via a transpetrosal approach, the patient has thus far been disease free for 7 years. The tumor tissue was composed of small cells with uniform round nuclei and minimal identifiable cytoplasm. Homer-Wright rosettes were frequently observed. Immunohistochemical studies demonstrated a positive reaction to HBA-71, which recognizes the cell surface glycoprotein p30/32, a product of the MIC2 gene. Both the clinical and immunohistochemical characteristics of this tumor are consistent with a diagnosis of peripheral PNET, which is genetically distinct from the more common intracranial PNET. ( info)

20/714. Clinical features of congenital absence of the superior oblique muscle as demonstrated by orbital imaging.

    INTRODUCTION: Absence of an extraocular muscle was considered rare when demonstrable only by surgical exploration or necropsy. This study presents advances in orbital imaging to correlate clinical findings with absence of the superior oblique (SO) muscle. methods: We performed high-resolution coronal orbital imaging by magnetic resonance imaging (222 orbits) or computerized radiographic tomography (32 orbits) in 127 patients with strabismus. We reviewed the histories and ocular motility examinations in patients who had absence of 1 or both SO muscles. Findings were compared with patients who were clinically diagnosed with SO palsy but had demonstrable SO muscles on orbital imaging. RESULTS: SO muscles were absent in 6 patients. All had histories suggesting congenital strabismus. In patients old enough for quantitative testing who had unilateral SO muscle absence, visual acuity was a least 20/25 in all and stereopsis was better than 80 arc/s in one. Three patients were orthotropic in primary position. Five patients with unilateral SO muscle absence had clinical findings variably consistent with SO palsy, whereas a sixth patient with Duane syndrome had clinically unsuspected bilateral SO muscle absence. Versions and patterns of hypertropia in patients with SO muscle absence overlapped findings of 20 patients with SO palsy but demonstrable SO muscles. CONCLUSIONS: Imaging can frequently demonstrate absence of the SO muscle in patients with SO palsy. Such patients may have good vision and stereopsis and clinical findings indistinguishable from SO palsy without absence of the SO muscle. Orbital imaging should be considered in the evaluation of congenital SO palsy to facilitate planning of effective surgical correction. ( info)
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