Cases reported "Cranial Nerve Diseases"

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1/18. Chronic steadily progressive central and peripheral predominantly motor demyelination, involving the cranial nerves, responsive to immunoglobulins.

    The association of central and peripheral demyelination was reported previously. Most of the cases refer to central chronic relapsing demyelination with clinical criteria for multiple sclerosis associated with later signs of peripheral nerve involvement. Other authors, described central lesions in patients with chronic inflammatory demyelinating polyneuropathy and in guillain-barre syndrome, as a seldom occurrence. We report a patient in which a chronic steadily progressive central and peripheral predominantly motor nervous system demyelination, involving the cranial nerves, was identified. The patient improved after intravenous immunoglobulin suggesting an immune-mediated mechanism. To our knowledge this presentation was not described before.
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ranking = 1
keywords = multiple sclerosis, sclerosis
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2/18. Cranial polyneuropathies in multiple sclerosis: case report and literature review.

    Although cranial neuropathies are common disorders in multiple sclerosis patients, multiple cranial nerve involvement is an unusual occurrence. Correlation of clinical symptoms with magnetic resonance imaging evidence of demyelinating central nervous system lesions can confirm the diagnosis. The authors report on the case of a 43-year-old woman who initially was thought to have suffered a brainstem infarct but, in fact, had developed multiple cranial nerve functional deficits. Treatment of multiple sclerosis remains primarily supportive in nature, with corticosteroids used for acute exacerbations and chronic progression.
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ranking = 6
keywords = multiple sclerosis, sclerosis
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3/18. Palatal tremor, progressive multiple cranial nerve palsies, and cerebellar ataxia: a case report and review of literature of palatal tremors in neurodegenerative disease.

    We describe a patient with an unusual clinical presentation of progressive multiple cranial nerve palsies, cerebellar ataxia, and palatal tremor (PT) resulting from an unknown etiology. magnetic resonance imaging showed evidence of hypertrophy of the inferior olivary nuclei, brain stem atrophy, and marked cerebellar atrophy. This combination of progressive multiple cranial nerve palsies, cerebellar ataxia, and PT has never been reported in the literature. We have also reviewed the literature of PT secondary to neurodegenerative causes. In a total of 23 patients, the common causes are sporadic olivopontocerebellar atrophy (OPCA; 22%), Alexander's disease (22%), unknown etiology (43.4%), and occasionally progressive supranuclear palsy (4.3%) and spinocerebellar degeneration (4.3%). Most patients present with progressive cerebellar ataxia and approximately two thirds of them have rhythmic tremors elsewhere. ear clicks are observed in 13% and evidence of hypertrophy of the inferior olivary nucleus in 25% of the patients. The common neurodegenerative causes of PT are OPCA/multiple system atrophy, Alexander's disease, and, in most of them, the result of an unknown cause.
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ranking = 120.59513392731
keywords = progressive multiple
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4/18. syringomyelia and complex regional pain syndrome as complications of multiple sclerosis.

    OBJECTIVE: To describe a patient from Southeast asia with the optic-spinal phenotype of multiple sclerosis who developed syringomyelia and resultant complex regional pain syndrome (formerly named reflex sympathetic dystrophy). DESIGN: Case report. SETTING: Department of neurology at a tertiary care hospital in the Republic of singapore. PATIENT: A 53-year-old Chinese woman with a history of optic neuritis developed an episode of left hemiparesis leading to a diagnosis of multiple sclerosis. Serial neuroimaging studies revealed an active demyelinating plaque in the cervical area that later progressed into a syrinx. Over a period of 1 year she also developed signs of sympathetic dysfunction including horner syndrome of the left eye and complex regional pain syndrome in the left hand. CONCLUSIONS: A case of the optic-spinal phenotype of multiple sclerosis that is commonly observed in Southeast asia is described. This characteristically tissue-destructive form of multiple sclerosis resulted in syringomyelia complicated by a complex regional pain syndrome. Possible pathogenic mechanisms for these associations are discussed.
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ranking = 8
keywords = multiple sclerosis, sclerosis
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5/18. Peripheral third cranial nerve enhancement in multiple sclerosis.

    Cranial nerve III dysfunction in multiple sclerosis (MS) is uncommon. Seven cases of isolated cranial nerve III paresis associated with MS have been reported in the English-language literature. MR imaging was obtained in five cases demonstrating lesions within the midbrain. We present the detailed clinical and MR imaging findings of a young woman with MS and an isolated, painful pupil involving complete left cranial nerve III palsy. Initial MR imaging showed isolated enhancement of the cisternal portion of the cranial nerve III, suggesting that peripheral nervous system involvement may develop as part of the disease process in some patients with MS.
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ranking = 5
keywords = multiple sclerosis, sclerosis
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6/18. Acute VIth cranial nerve dysfunction in multiple sclerosis. Evaluation by magnetic resonance imaging.

    VIth nerve palsy is not frequently considered a presenting sign of multiple sclerosis (MS); however, MS has been documented as a fairly common cause of VIth nerve dysfunction. In the present study we have evaluated the clinical features and magnetic resonance imaging (MRI) findings in four MS patients with acute VIth nerve palsy. diplopia as a result of acute VIth nerve palsy was the prominent symptom leading to the diagnosis of MS in all of the individuals. Other signs specifically localizing to the ipsilateral brainstem were absent in these patients. Cranial MRI revealed multiple white matter lesions with a periventricular predominance in all four patients and pontine white matter lesions in three of the patients. These lesions were either adjacent to the VIth nerve nucleus or involved the fasciculus of the VIth nerve or both.
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ranking = 5
keywords = multiple sclerosis, sclerosis
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7/18. Spinal and cranial hypertrophic neuropathy in multiple sclerosis.

    Two patients with multiple sclerosis developed symptomatic chronic inflammatory demyelinating polyneuropathy with massive spinal or cranial nerve hypertrophy revealed by neuroimaging. sural nerve biopsy in one showed only moderate demyelination, axonal loss, and onion-bulb formation, illustrating dichotomy between severe proximal and milder distal nerve involvement. patients with coexistent central and peripheral demyelination usually are symptomatic from dysfunction at one site or the other, but not from both. Our patients showed minimal response to steroids, intravenous immunoglobulin, or azathioprine. These cases suggest that the mechanism of disease in symptomatic central and peripheral demyelination may differ from that of disease in only one region, and that optimal therapy in this situation must be explored further.
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ranking = 5
keywords = multiple sclerosis, sclerosis
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8/18. Optic chiasmal neuritis.

    In four of six patients with clinical optic chiasmal neuritis, MRI demonstrated abnormalities of the chiasm. Optic chiasmal neuritis may be the initial manifestation of multiple sclerosis, a reflection of established CNS demyelination, or an isolated clinical finding.
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ranking = 1
keywords = multiple sclerosis, sclerosis
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9/18. Neurological complications of systemic sclerosis--a report of three cases and review of the literature.

    We report three cases of systemic sclerosis demonstrating four different neurological complications: trigeminal neuropathy, peripheral neuropathy, carpal-tunnel syndrome and prolonged response to local anaesthesia. A review of the literature reveals a wide range of neurological abnormalities associated with systemic sclerosis. When they occur, these are often presenting features.
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ranking = 0.31394261144316
keywords = sclerosis
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10/18. Isolated trigeminal sensory neuropathy: a heterogeneous group of disorders.

    Neuropathy affecting the face is a serious sign that warrants thorough and often multidisciplinary investigation. patients with isolated trigeminal sensory neuropathy (TSN) should be monitored for connective tissue disease, and some may develop multiple sclerosis or paroxysmal trigeminal neuralgia. A patient with chronic isolated TSN and sjogren's syndrome, Raynaud's phenomenon, arthropathy, vitiligo, and evidence of other immune dysregulation is presented. The literature is reviewed with respect to the heterogeneous etiology of TSN, and an attempt is made to identify subgroups and to evaluate prognosis from details of the history and systemic disorder associations.
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ranking = 1
keywords = multiple sclerosis, sclerosis
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