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Query: UMLS:C0235394 (wasting)
 8,040 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A kinship is described in which there was slowly progressive wasting and weakness of the muscles of the upper and occasionally of the lower limbs. Some members had hyperreflexia. There were no sensory abnormalities. Electrophysiological study suggested the presence of motor peripheral polyneuropathy. The condition appeared to be inherited as an autosomal dominant. The disorder does not appear typical of any of the known hereditary polyneuropathies and it is possible that it may represent a unique hereditary, dominantly motor, polyneuropathy. The significance of the hyperreflexia is uncertain, but raises the possibility of minor central involvement as well as peripheral neuropathy.
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PMID:Hereditary motor peripheral neuropathy predominantly affecting the arms. 18 Feb 64

Five cases of a chronic neuromuscular syndrome consisted of muscular aching and sometimes burning pain, fasciculations, cramps, fatigue, and occasional paresthesia. The disorder affected the legs and, less commonly, the girdle, trunk, and arm muscles. The symptoms were enhanced by physical activity and were usually improved by rest. Neither muscular wasting nor weakness was found, although the condition was present for an average of 4.7 years and, in one patient, as long as 10 years. Electrophysiologic studies showed motor abnormalities indicative of axonal degeneration and muscle fiber denervation, most marked in the legs. Light microscopy of skeletal muscle and spinal cord in one case disclosed evidence of mild denervation atrophy in muscle, but no loss of anterior horn cells. The findings are compatible with a benign polyneuropathy.
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PMID:The muscular pain-fasciculation syndrome. 56 28

Two siblings and a third child exhibited a syndrome of progressive muscular weakness and wasting, closely resembling Werdnig-Hoffmann's disease. Autopsy of one of the siblings and the third child showed nearly total absence of myelin sheaths in the cranial and spinal nerve roots, relative preservation of axons, and normal neurons in the motor cranial nerve nuclei and anterior spinal gray matter. The mother of the siblings had bilateral pes cavus, and the father of the third child had a sensory-motor polyneuropathy dating to childhood, associated with pes cavus and scoliosis. The disorder in these children and in a few similar cases in the literature shares some features of Charcot-Marie-Tooth disease and the hypertrophic neuropathy of Dejerine-Sottas, but it is difficult to classify as either of these familial neuropathies as presently defined. Elevation of cerebrospinal fluid protein is a useful finding in distinguishing such children from patients with Werdnig-Hoffmann's disease.
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PMID:Chronic polyradiculoneuropathy of infancy. A report of three cases with familial incidence. 94

The pathology of primary familia amyloidosis with polyneuropathy is described on the basis of post-mortem examination of six cases from Northern Sweden. Clinically the disease is characterized by progressive sensory and motor disturbances with loss of sensation, muscular wasting and flaccid paralysis. Impotence, urinary bladder dysfunction, motility disturbances of the gastro-intestinal tract and postural hypotension indicate affection of the autonomic nervous system as well. Malabsorption, cardiac insufficiency and vitreous opacites also occur. As regards the distribution of amyloid, the following findings seemed to be characteristic. Usually there were no gross lesions indicating the amyloid disease. Histopathologically, amyloid deposits were observed in great extent in the peripheral nervous system and in various parts of the peripheral autonomic nervous system as well. It occurred extensively in the walls of blood vessels of various calibres, in the perivascular collagenous connective tissue and adjacent to the smooth musculature. Amyloid deposition was also found more or less abundantly in various other organs and tissues. No deposits, however, or only insignificant amounts, were found in the central nervous system, either in the parenchyma of the liver, in the islets of Langerhans, or in the bone marrow. Clinical manifestations seemed to be related to the local deposition of the amyloid substance. Our clinical and pathological findings in this particular type of familial amyloidosis conformed mostly to those previously described.
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PMID:Postmortem findings in primary familial amyloidosis with polyneuropathy. 115 99

A case of a young man who presented symptoms and clinical signs of polyneuropathy that occurred in connection with recurrent hypoglycemic episodes is reported. The hypoglycemia was probably caused by a pancreatic islet tumor. There were symmetric weakness and wasting of hands and feet, absent tendon reflexes and 'glove and stocking' loss of sensation. Electromyographic studies showed denervation potentials with slight reduction of nerve conduction velocities. Sural nerve biopsy studied by optic and electronic microscopy showed axonal degeneration without signs of demyelination or remyelination. There are only 30 similar cases reported in the literature. According to experimental findings, the authors believe that glucopenia is the mechanism responsible for the development of the neuropathy, and that at present time there is no evidence for a direct insulin effect.
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PMID:[Hypoglycemic polyneuropathy: report of a case with insulinoma]. 255 83

Nineteen patients developed polyneuropathy complicating critical illness. They had been admitted to a critical care unit following intubation for cardiac or pulmonary disease and had developed sepsis and multiple organ failure. Approximately one month following intubation, failure to wean from the ventilator and limb weakness prompted neurological referral. Examination disclosed weakness and wasting of muscles and impaired tendon reflexes in most, but not all, patients. Electrophysiological studies in 17 patients revealed attenuated compound muscle and sensory nerve action potential amplitudes and widespread denervation on needle electromyography. Autopsy in 9 patients who died of their critical illness revealed widespread primary axonal degeneration of motor and sensory fibres, with extensive denervation atrophy of limb and respiratory muscles. Survivors recovered from the polyneuropathy three to six months following discharge. Seventeen of the patients were segregated by electrophysiological criteria into mild (8) and severe (9) polyneuropathy categories. An analysis of these two groups failed to reveal putative metabolic, drug, nutritional or toxic factors that might be responsible for the polyneuropathy. Our studies suggest that the mechanism may be a fundamental defect, still unknown, which causes dysfunction of all organ systems in this syndrome.
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PMID:Critical illness polyneuropathy. A complication of sepsis and multiple organ failure. 365 96

A 52-year-old woman presented with increasing pain, weakness, and paraesthesiae of four months' duration in the lower limbs. She suffered from chronic obstructive airways disease and hypertension. Neurological examination revealed wasting of the quadriceps muscles, weakness of the lower limbs, and absent ankle jerks. The sensory examination was normal. Full blood count, ESR, biochemical, immunological, and viral studies, urinary heavy metal assays, and cerebrospinal fluid examination were normal. Nerve conduction studies were consistent with a sensorimotor neuropathy, and electromyographic sampling was consistent with acute denervation. A sural nerve biopsy showed axonal degeneration and segmental demyelination. One month after admission, she developed carbon dioxide retention. Her weakness spread to affect the upper limbs, and she could not be resuscitated after a cardiac arrest three months after admission. General autopsy examination revealed bronchopneumonia. Neuropathological examination showed a lymphocytic infiltrate in the nerve roots of the cauda equina, the lumbosacral plexus, and the sural and vagal nerves. Increased cellularity and collagen were evident in these nerves. A diagnosis of chronic inflammatory polyneuropathy was made. The neuropathology of this entity is discussed.
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PMID:Neuropathological findings in a case of chronic inflammatory polyneuropathy. 384 15

A syndrome of peripheral polyneuropathy associated with islet cell tumors and hypoglycemia has been reported in 28 patients. Despite varying features in these patients, the clinical characteristics of this syndrome are remarkably similar. These consist of the development of a sensorimotor neuropathy during a protracted course of recurrent severe hypoglycemia, related to underlying insulinoma. Cerebral symptoms dominate the clinical picture and a predominantly or entirely motor, distal and symmetric, peripheral neuropathy ensures. Upper limb involvement is more frequent, accompanied by severe weakness and distal wasting, usually without fasciculations. Painful distal paresthesias without objective sensory loss are characteristic. Direct relationship to a single hypoglycemic insult is often absent. This report describes the clinical features and laboratory investigation of a new case with this condition, reviews the literature and discusses the syndrome with special regard to the etiology.
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PMID:Hypoglycemic peripheral neuropathy in association with insulinoma: implication of glucopenia rather than hyperinsulinism. Case report and literature review. 627 47

A 77-year-old male was hospitalized with a 6-day history of severe posterior cervical pain and headache. An initial lumbar puncture revealed polymorphonuclear pleocytosis indicating pyogenic meningitis. The blood and throat cultures on admission were positive for Staphylococcus aureus. A combination of ceftriaxone and ABPC, both of which were sensitive to the pathogen, were intravenously administered for one month, by which he responded well as to the meningitis. However, he was found to have persistent neck pain, and wasting and weakness of the shoulder girdles, which were associated with polyneuropathy-like sensory loss. Neuroimaging studies disclosed a partial destruction of the odontoid process, subluxation of the atlantoaxial joint, MRI evidence of an inflammatory pseudomass formation dorsal to the odontoid process and an increased uptake of radionuclide on bone scanning, all of which were indicative of osteomyelitic processes at the region of the axis; i.e., the meningitis was thought to be secondary to osteomyelitis of the odontoid process and hence the foramen magnum syndrome characteristic to this particular case was explained by the compression of the cervicomedullary junction due to the pseudomass. To our knowledge, this is the first case of primary odontoid osteomyelitis to be reported in Japan.
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PMID:[Acute bacterial meningitis and foramen magnum syndrome as a presentation of odontoid osteomyelitis. A case report]. 766 25

Multifocal motor neuropathy, which mimics lower motor neuron disease, is a rare and curious demyelinating neuropathy characterised by slowly progressive, asymmetric limb weakness within the distribution of individual peripheral nerves, wasting, cramps, fasciculations and rare sensory involvement, but without upper motor neuron signs. The cardinal feature and primary pathophysiological basis for the weakness is the multifocal motor conduction block which remains stable for years at the same site and is confined to motor axons. It is defined as > 50% reduction in both the CMAP and the negative peak area on proximal stimulation, as compared with the distal stimulus response without any change in the negative peak duration. Nerves at the site of the conduction block show demyelination, endoneural edema, rudimentary onion bulbs and lymphocytic inflammation. Sensory nerves may show mild demyelination, axon loss and lymphocytic inflammation. The majority of patients shows elevated titers of anti-glycolipid antibodies, which may block the Na+ channels, produce demyelination or interfere with remyelination. However, their role in the pathogenesis of multifocal motor neuropathy remains uncertain. Multifocal motor neuropathy is regarded as the predominantly motor variant of chronic inflammatory demyelinating polyneuropathy and can be treated best with immunoglobulins and cyclophosphamide.
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PMID:[Multifocal-motor neuropathy and motor neuropathy with multifocal conduction block (Lewis-Sumner syndrome)]. 778 76


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