1/198. 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 = nervous system (Clic here for more details about this article) |
2/198. styrene-induced peripheral neuropathy.CASE REPORT: styrene is a colorless, oily liquid most commonly found in paints, plastics, and resins. Like many solvents, styrene can cause intoxication and central nervous system depression when inhaled in high concentrations for extended periods. Rarely, styrene has been implicated as a cause of peripheral neuropathy. We describe a case of a previously healthy 57-year-old man who developed signs and symptoms consistent with a peripheral neuropathy after applying a fiberglass resin to the inside of a septic tank over a 2-day period. Nerve conduction tests verified examination findings. styrene exposure should be minimized through the use of respirators and protective clothing to prevent this type of toxicity.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
3/198. Congenital muscular dystrophy with central and peripheral nervous system involvement in a Belgian patient.We report a patient with congenital muscular dystrophy (CMD), developmental brain defects, and peripheral neuropathy. Marked hypotonia and plagiocephaly were noted at birth. failure to thrive, generalized muscle weakness and wasting, absent deep tendon reflexes, partial seizures, and secondary microcephaly developed. brain MRI showed a large area of cortical dysplasia, a thin but complete corpus callosum, and diffuse ventriculomegaly. Nerve conduction velocities were slow and creatine kinase levels only mildly elevated. Muscle biopsy showed dystrophic features with normal merosin, sarcoglycan, and dystrophin immunostaining. The Japanese Fukuyama CMD founder mutation was not detected. This is the first report of a patient with merosin-positive CMD, cobblestone lissencephaly, and demyelinating peripheral neuropathy.- - - - - - - - - - ranking = 4keywords = nervous system (Clic here for more details about this article) |
4/198. Organophosphate neuropathy due to methamidophos: biochemical and neurophysiological markers.Neuropathy target esterase (NTE), the putative target enzyme for organophosphate induced delayed polyneuropathy (OPIDP), can be measured in lymphocytes but has rarely been assessed in acute human poisoning. serum autoantibodies to nervous system proteins develop in hens poisoned with neuropathic insecticides and also have not been studied after human poisoning. Serial lymphocyte NTE (LNTE) was measured in a 16-year-old boy after acute poisoning with methamidophos for evaluation as a predictor of subsequent neuropathy. The profiles of serum autoantibodies to neurofilament triplet proteins, myelin basic protein, and glial fibrillary acidic protein were measured in order to characterize changes occurring as a result of OPIDP. Clinical neuropathy characterized by steppage gate and profound lower extremity weakness, decreased grip and pinch strength, and decreased ulnar and absent tibial compound muscle action potentials developed 2 weeks following poisoning. Sensory examination and nerve conduction studies were normal. On day 3 following poisoning LNTE was depressed (77% compared with subsequent baseline enzyme activity). Marked increases in serum immunoglobulin g (IgG) autoantibodies to glial fibrillary acidic protein and to neurofilament 200 were observed after the development of OPIDP. We conclude that inhibition of lymphocyte NTE is predictive of subsequent OPIDP. serum autoantibody titers to nervous system proteins may be useful markers of neuropathy.- - - - - - - - - - ranking = 2keywords = nervous system (Clic here for more details about this article) |
5/198. Anterior lumbosacral radiculopathy after intrathecal methotrexate treatment.Intrathecal chemotherapy with methotrexate or cytosine arabinoside is the standard approach to prophylaxis and treatment of central nervous system leukemia in children. Progressive paraplegia, one of the devastating neurologic complications related to this mode of treatment, has been attributed to spinal cord toxicity. Reported are three children who developed progressive paraparesis after intrathecal methotrexate administration followed by complete or partial recovery. gadolinium enhancement of anterior lumbosacral spinal nerve roots was demonstrated in all three patients, and an elevation of cerebrospinal fluid immunoglobulin g synthesis was evident in two patients with more severe symptoms. The clinical data suggest that anterior lumbosacral radiculopathy is also a type of neurologic complication associated with intrathecal methotrexate treatment.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
6/198. Polyneuropathy as initial manifestation of systemic sclerosis (scleroderma).We report the first case of a young female patient who developed a sensory-motor polyneuropathy, without any skin or internal involvement characteristic of SSc, but with a serological positivity of antitopoisomerase I antibodies. After 4 years she developed a rapid skin tightening with lung involvement, in a full blown picture of the diffuse subset of SSc. The case suggests that the peripheral nervous system deserves more attention, in particular in the earliest phase of SSc.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
7/198. Subacute sensory neuropathy associated with Epstein-Barr virus.A 35-year-old man experienced severe sensory loss, pseudoathetosis, and areflexia during recovery from a severe viral illness. Sensory nerve action potentials were absent, motor conduction velocities were mildly slowed, and blink reflexes were normal. magnetic resonance imaging (MRI) revealed abnormal signal within the central and dorsal aspects of the thoracic cord. Acute and convalescent Epstein-Barr virus (EBV) titers suggested EBV as the etiology. Subacute sensory neuropathy, with peripheral and central nervous system involvement, is a rare complication of EBV infection.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
8/198. Angiotropic large B-cell lymphoma with clinical features resembling subacute combined degeneration of the cord.Angiotropic large cell lymphoma is a rare neoplastic disorder associated with a high mortality. The hallmark of the disease is lymphoid proliferation confined to the intravascular compartment without local tissue or vessel wall infiltration [1]. This feature is so striking that the disease was originally thought to arise from endothelial tissue and early cases were described as malignant angioendotheliomatosis. However, application of immunohistochemical methods for detection of lymphoid markers such as the CD45 and CD20 cell surface markers has confirmed its lymphoid origin, usually of B-cell lineage [2]. Clinical manifestations of the disease are protean and are due to multifocal medium and small vessel occlusion by tumour cells [3]. Characteristic sites of involvement are skin and central nervous system and although an ante-mortem diagnosis can be made from a biopsy specimen, it is often unsuspected [4]. We present a case of angiotropic large B-cell lymphoma in a 74-year-old man who presented with urinary symptoms and had a neurological picture resembling subacute combined degeneration of the cord.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
9/198. Bilateral phrenic neuropathy as a presenting feature of multifocal motor neuropathy with conduction block.Diaphragmatic paralysis has previously been reported as a result of diverse pathologic processes involving the peripheral nervous system. We report the clinical history, physical findings, and antibody profile of an atypical case of multifocal motor neuropathy with conduction block initially presenting with respiratory failure secondary to bilateral phrenic neuropathy.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
10/198. role of neurophysiologic evaluation in diagnosis.The electrodiagnostic evaluation assesses the integrity of the lower-motor-neuron unit (i.e., peripheral nerves, neuromuscular junction, and muscle). Sensory- and motor-nerve conduction studies measure compound action potentials from nerve or muscle and are useful for assessing possible axon loss and/or demyelination. Needle electromyography measures electrical activity directly from muscle and provides information about the integrity of the motor unit; it can be used to detect loss of axons (denervation) as well as reinnervation. The electrodiagnostic examination is a useful tool for first detecting abnormalities and then distinguishing problems that affect the peripheral nervous system. In evaluating the patient with extremity trauma, it can differentiate neurapraxia from axonal transection and can be helpful in following the clinical course. In patients with complex physical findings, it is a useful adjunct that can help discriminate motor neuron disease from polyneuropathy or myeloradiculopathy due to spondylosis.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
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