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.- - - - - - - - - - ranking = 1keywords = chronic inflammatory demyelinating, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
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.- - - - - - - - - - ranking = 0.013491611496973keywords = demyelinating (Clic here for more details about this article) |
3/18. 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.- - - - - - - - - - ranking = 0.013491611496973keywords = demyelinating (Clic here for more details about this article) |
4/18. Phe 84 deletion of the PMP22 gene associated with hereditary motor and sensory neuropathy HMSN III with multiple cranial neuropathy: clinical, neurophysiological and magnetic resonance imaging findings.Hereditary motor and sensory neuropathy (HMSN) is a heterogeneous group of peripheral neuropathies which are diagnosed on the basis of clinical, electrophysiological and neuropathological findings. Among the hypertrophic demyelinating neuropathies, HMSN III is the most severe. It is often associated with de novo mutations in the genes encoding for peripheral myelin proteins. While peripheral nerve hypertrophy is an expected finding in HMSN III, cranial nerve hypertrophy is exceptional. Here we describe a mutation in the PMP22 gene in a 19-year-old man with infantile onset of sensory motor polyneuropathy without family history and multiple cranial nerve hypertrophy shown by cranial magnetic resonance imaging.- - - - - - - - - - ranking = 0.013491611496973keywords = demyelinating (Clic here for more details about this article) |
5/18. Cranial and peripheral neuropathies.An acute polyneuropathy developing over days to several weeks is most likely to be guillain-barre syndrome or a toxic neuropathy, although vasculitis can also present acutely. This presentation should be referred immediately for further investigation. A subacute to chronic (ie, developing over months) neuropathy with significant proximal weakness and prominent loss of reflexes is highly suggestive of chronic inflammatory demyelinating polyradiculoneuropathy. If there is a clear stepwise onset of symptoms, suggestive of multiple mononeuropathies, or significant asymmetry, vasculitic neuropathy should be considered, even in the absence of systemic vasculitis. Idiopathic chronic axonal neuropathy is an indolent, predominantly sensory neuropathy that typically occurs in older patients. Neuropathies occurring in young or middle age or with more subacute onset always warrant further investigation.- - - - - - - - - - ranking = 1keywords = chronic inflammatory demyelinating, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
6/18. Childhood-onset chronic inflammatory demyelinating polyradiculoneuropathy with cranial nerve involvement.A 17-year-old male presented with chronic diplopia and generalized motor weakness. He was previously diagnosed with chronic inflammatory demyelinating polyradiculoneuropathy and acute disseminated encephalomyelitis in childhood. Cranial magnetic resonance imaging (MRI) revealed a rarely reported finding of thickening and enhancement of multiple cranial nerves. Nerve conduction studies and electromyography showed peripheral nerve demyelination with axonal involvement. There was improvement in the clinical examination, MRI, and electrophysiologic studies after combined corticosteroid and plasma exchange therapy. We review the clinical presentation, course, and response to therapy among children with chronic inflammatory demyelinating polyradiculoneuropathy, with specific emphasis on the frequency and pattern of cranial nerve involvement.- - - - - - - - - - ranking = 6keywords = chronic inflammatory demyelinating, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
7/18. Multifocal demyelinating motor neuropathy: cranial nerve involvement and immunoglobulin therapy.We report two patients with multifocal demyelinating motor neuropathy whose symptoms improved after immunoglobulin infusion. Clinical signs included atrophy of the tongue and limb muscles, closely resembling that of motor neuron disease. Nerve conduction studies revealed multiple sites of motor conduction block without sensory abnormalities, suggesting selective involvement of motor fibers. Careful documentation of conduction block is required to diagnose this entity, which may be treated with immunoglobulin therapy.- - - - - - - - - - ranking = 0.067458057484865keywords = demyelinating (Clic here for more details about this article) |
8/18. 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.- - - - - - - - - - ranking = 1keywords = chronic inflammatory demyelinating, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
9/18. Encephalopathy and peripheral neuropathy following diethylene glycol ingestion.The authors report a 24-year-old man who developed encephalopathy and rapid quadriplegia following ingestion of a solution containing diethylene glycol (DEG). As quadriparesis evolved, motor response amplitudes were markedly reduced with preserved conduction velocities. Studies during clinical recovery revealed marked motor conduction velocity slowing and prolonged distal latencies. These data indicate that DEG intoxication may cause a primary acute axonal sensorimotor polyneuropathy with demyelinating physiology during recovery.- - - - - - - - - - ranking = 0.013491611496973keywords = demyelinating (Clic here for more details about this article) |
10/18. Bulbar encephalitis complicating trigeminal zoster in the acquired immune deficiency syndrome.A 30-year-old homosexual man with the acquired immune deficiency syndrome and a recent history of zoster involving the mandibular division of the right trigeminal nerve was found at autopsy to have a predominantly demyelinating lesion restricted to the ipsilateral spinal trigeminal tract and nucleus. Cowdry A inclusions were readily apparent in the nuclei of numerous glia and isolated neurons, and inclusion-bearing cells were immunoreactive with an antiserum to the varicella zoster virus (VZV). This represents the first demonstration that zoster-associated trigeminal encephalitis is the result of brainstem invasion by VZV. The restricted topography of this lesion implicates axonal transport in its pathogenesis.- - - - - - - - - - ranking = 0.013491611496973keywords = demyelinating (Clic here for more details about this article) |
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