1/23. 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) |
2/23. Motor and sensory demyelinating mononeuropathy multiplex (multifocal motor and sensory demyelinating neuropathy): a separate entity or a variant of chronic inflammatory demyelinating polyneuropathy?We report 16 patients with motor and sensory demyelinating mononeuropathy multiplex (MSDMM) or multifocal motor and sensory demyelinating neuropathy (MMSDN). These patients had the clinical pattern of motor and sensory mononeuropathy multiplex, electrophysiological evidence of demyelination including conduction block, and segmental demyelination in the sural nerve biopsy. Sixty per cent of patients had high CSF protein. Eighty per cent of patients showed good responsiveness to steroid treatment. Unlike multifocal motor neuropathy (MMN), MSDMM is characterized by a shorter course, sensory deficits and sensory nerve conduction abnormalities, absence of GM1 antibody in most patients tested, and a good response to steroid therapy. We believe that MSDMM represents a variant of chronic inflammatory demyelinating polyneuropathy (CIDP) and an intermediate link between CIDP and MMN.- - - - - - - - - - ranking = 5.1845656250903keywords = chronic inflammatory demyelinating, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
3/23. Anterior horn cell disease and olivopontocerebellar hypoplasia.To date, fewer than 30 cases of anterior horn cell disease with associated olivopontocerebellar hypoplasia have been reported. We describe five patients and review the literature on this uncommon disorder. In addition to a syndrome of progressive spinal muscular atrophy similar to that seen in Werdnig-Hoffmann disease, this disorder is characterised by hypoplasia of the olivary nuclei, pons, and cerebellum. Additional clinical features may include dysmorphism, abnormal eye movements, stridor, congenital joint contractures, and enlarged kidneys. Pontocerebellar hypoplasia may be associated with posterior fossa cystic malformations, cerebral atrophy, and a demyelinating neuropathy.- - - - - - - - - - ranking = 0.018456562509034keywords = demyelinating (Clic here for more details about this article) |
4/23. Mimic syndromes in sporadic cases of progressive spinal muscular atrophy.Described are patients initially diagnosed with progressive spinal muscular atrophy (PSMA), in whom further evaluation established another diagnosis. The authors prospectively investigated incident and prevalent cases of PSMA. Seventeen of 89 patients, after initial registration, were later excluded because reassessment revealed a diagnosis other than PSMA. In 11 of the 17 patients with a revised diagnosis, a potential treatment was available: multifocal motor neuropathy (7), chronic inflammatory demyelinating polyneuropathy (2), inflammatory myopathy (1), and MG (1). Other misdiagnoses included myopathy, syringomyelia, ALS, idiopathic chronic axonal polyneuropathy, and idiopathic brachial plexus neuropathy. One patient with a possible herniated lumbar disk recovered spontaneously.- - - - - - - - - - ranking = 1keywords = chronic inflammatory demyelinating, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
5/23. A variant of multifocal motor neuropathy with acute, generalised presentation and persistent conduction blocks.OBJECTIVE: Multifocal motor neuropathy with persistent conduction blocks is classically described as a chronic neuropathy with progressive onset, and acute forms have not previously been characterised. We report four cases of severe motor impairment with acute and generalised onset and with persistent motor conduction blocks. patients AND RESULTS: An acute tetraparesis with diffuse areflexia but little or no sensory disturbance was the clinical picture. Serial electrophysiological tests showed persistent multifocal motor conduction blocks with absent F waves in most tested motor nerves. No or minor abnormalities of the sensory nerve action potentials were observed. cerebrospinal fluid contained normal or mildly increased protein levels (<1 g/l) without cells. campylobacter jejuni serology was negative in three patients and consistent with past infection in one patient. Anti-ganglioside antibodies were positive in three patients. A five day course of intravenous immunoglobulins produced nearly complete symptom resolution in three patients and was ineffective in one patient. CONCLUSION: Because of the persistence of multifocal motor conduction blocks for several weeks or months as the isolated electrophysiological feature, these cases could not be consistent with guillain-barre syndrome or chronic inflammatory demyelinating polyneuropathy. They suggest an original variant of multifocal motor neuropathy with an acute and generalised initial presentation and persistent motor conduction blocks affecting all four limbs.- - - - - - - - - - ranking = 1keywords = chronic inflammatory demyelinating, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
6/23. Corticospinal tract MRI hyperintensity in X-linked charcot-marie-tooth disease.In X-linked hereditary demyelinating neuropathies (CMTX), caused by mutations in Connexin 32, mild subclinical CNS involvement is not unusual. We present a young male patient suffering from genetically proven CMTX who presented with permanent bilateral corticospinal tract hyperintensities in cranial MRI -- a finding previously described to be characteristic for amyotrophic lateral sclerosis. MRI seems to be able to visualize corticospinal tract abnormalities, even if subclinical, in CMTX.- - - - - - - - - - ranking = 0.018456562509034keywords = demyelinating (Clic here for more details about this article) |
7/23. High-dose cyclophosphamide without stem cell rescue for refractory multifocal motor neuropathy.patients with multifocal motor neuropathy (MMN) respond to intravenous immune globulin (IVIg) and cyclophosphamide, although the benefit is not sustained. High-dose cyclophosphamide can induce a remission in patients with autoimmune diseases, including chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). We describe a patient with refractory MMN who improved with high-dose cyclophosphamide (50 mg/kg for 4 days) without stem cell rescue. Following treatment she discontinued IVIg. At a 6-month examination, her strength had improved and she had regained the ability to write. High-dose cyclophosphamide may be a successful treatment for patients with MMN refractory to other therapies.- - - - - - - - - - ranking = 1keywords = chronic inflammatory demyelinating, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
8/23. Multifocal motor neuropathy with conduction block associated with metastatic lymphoma of the nervous system.Peripheral neuropathies occur in 5% of patients with Non-Hodgkin lymphoma and represent the effects of therapy, direct compression or nerve infiltration by tumor, or paraneoplastic effects. Multifocal motor neuropathy with conduction block (MMNCB) is a rare demyelinating disorder of unknown etiology characterized by progressive, distal, asymmetric weakness mostly of the upper limbs with minimal or no sensory loss. We report a patient, who developed MMNCB at the time of isolated CNS relapse from a diffuse large B-cell lymphoma. Marked neurological improvement was achieved using intravenous immunoglobulin treatment. To our knowledge, MMNCB has thus far not been described as part of the spectrum of lymphoma-related peripheral neuropathies.- - - - - - - - - - ranking = 0.018456562509034keywords = demyelinating (Clic here for more details about this article) |
9/23. dysarthria of motor neuron disease: longitudinal measures of segmental durations.motor neuron disease encompasses a group of terminal, demyelinating diseases affecting upper- and lower-motor neurons and producing muscular weakness resulting in a flaccid, spastic, or spastic-flaccid dysarthria of speech. The present study presents measurements of the temporal-acoustic characteristics of dysarthria in three subjects with motor neuron disease over a two-year recording period. Changes seen over the course of the disease varied by type of motor neuron disease, though all types demonstrated some degree of neutralization of the prevocalic VOT, target vowel duration, and postvocalic closure duration. These changes are discussed with relation to physical manifestation and progression of the disease.- - - - - - - - - - ranking = 0.018456562509034keywords = demyelinating (Clic here for more details about this article) |
10/23. Diphtheritic neuropathy.A 29-year-old Haitian man had a sore throat with a marked tonsillar exudate followed by the onset of increasingly blurred vision with swallowing difficulties and, then, a diffuse acute demyelinating neuropathy. cerebrospinal fluid showed moderate pleiocytosis and elevated protein content. Serological testing for diphtheria, but not for tetanus and poliomyelitis, was markedly positive. Other tests including HIV1 and 2, HTLV1 and lyme disease were negative. Diphtheritic neuropathy should be considered in the differential diagnosis of guillain-barre syndrome in nonvaccinated patients.- - - - - - - - - - ranking = 0.018456562509034keywords = demyelinating (Clic here for more details about this article) |
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