Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0004134 (ataxia)
 15,886 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Acrylamide, widely employed as a vinyl monomer in the polymer industry, is a potent neurotoxin to man and to animals. The cumulative effect of prolonged, low-level exposure to acrylamide monomer is the insidious development of a progressive peripheral neuropathy. Sensory symptoms begin in the hands and feet (numbness, pins and needles), certain reflexes are lost and, with severe exposure, muscle weakness and atrophy occur in the extremities. The peripheral neuropathy may be supplemented by symptoms indicative of central nervous system damage (ataxia, tremor, somnolence and mental changes). The neuropathologic basis for this clinical picture has been determined in cats. Here, chronic acrylamide intoxication produces selective peripheral and central nerve fiber degeneration. Degeneration first occurs in the extremities of long and large nerve fibers which later undergo a progressive, seriate proximal axonal degeneration known as dying-back. Especially vulnerable are sensory axons supplying Pacinian corpuscles and muscle spindles in the hindfoot toepads, while adjacent motor nerve axons die back later. Distal central nerve fiber degeneration is seen in the medulla and the cerebellum. The neurotoxic property of acrylamide is of practical concern in two areas. One major problem is the protection of factory workers engaged in the manufacture of acrylamide. A sensitive test of neurologic function in these individuals, i.e., touch sensation, based on the experimental observation of the exquisite vulnerability of Pacinian corpuscles in acrylamide intoxicated cats, is presently under consideration. The second area for concern is the exposure of the populace to minute amounts of neurotoxic acrylamide monomer which contaminate acrylamide polymers currently deployed in the environment. Federal restrictions on the maximum permitted exposure to acrylamide, based on a largely clinical study of acrylamide neurotoxicity conducted ten years ago, may require a re-evaluation in the light of recent advances which have pinpointed the initial sites of nerve fiber degeneration.
 ...
PMID:Nervous system degeneration produced by acrylamide monomer. 17 76

The first autopsy of a case of multiple sclerosis from the District of Hokuriku was reported. The patient, a 50-year-old house-wife, born in Toyama Prefecture, had noticed a paresthesia of her face, fatigue, numbness and weakness in the right limbs, dimness of vision and gait disturbance at ave 44. Furthermore, in the course of the disease, she had suffered from visual disorder, tetraplegia, hyperreflexia, pyramidal signs and cerebellar syndroms such as dysarthria, nystagmus, intention tremor and ataxia. She also showed symptoms of euphoria and dementia. After a course of six years she died of bronchopneumonia. Remissions and exacerbations were noted four times during her clinical history. Histopathologically, there were many recent and old demyelinating lesions of varying sizes and shapes in all parts of the central nervous system, namely the cerebrum, brainstem, spinal cord and optic nerve. In contrast to the clinical symptoms, the cerebellum itself revealed less plaques than the other areas of the brain. According to the observed distributions of the lesions, our case can be classified as belonging to the optico-cerebro-spinal type in the Ikuta and Zimmerman classification. The demyelinated lesions were characterized by a perivenular distribution of the plaques, lack of tissue necrosis, paucity of inflammatory reaction and marked fibrous gliosis of varying degrees.
 ...
PMID:First autopsy report of a multiple sclerosis case in Hokuriku District of Japan. 53 38

Developmental failure of the peripheral nervous system to form myelin is advanced as the probable mechanism of a severe neuropathy in young child. The hypothesis evolved from evaluation of clinical, electromyographic, and muscle biopsy studies at 9 months and 51/2 years of age and electron microscopic and biochemical studies of the sural nerve at the latter age. The clinical state was characterized by loss of sensation to modalities, usually ascribed as transmitted by large myelinated axons; those carried by smaller axons being relatively preserved. Thus, at the age of 51/2 years, ataxia due to absent peripheral orientation was the chief deficit. Muscle strength was decreased but still remarkable considering the histologically confirmed absence of myelin in intramuscular nerves and extremely low conduction velocities (2 to 3 m/sec) at both ages. Histological and ultrastructural features of the intramuscular and sural nerves included almost total lack of myelin sheaths, good preservation of axons, and marked proliferation of Schwann cells and their basement membranes with onion-bulb formation. The morphological findings correlated well with the absence of cholesterol esters and the presence of the normal myelin lipids in extremely small amounts in the lipid study of the sural nerve.
 ...
PMID:A case of congenital hypomyelination neuropathy. Clinical, morphological, and chemical studies. 87 Dec 59

Sixty-six hospitalized patients suffering from chronic methylmercury poisoning were examined in Baghdad during 1972. The poisoning was attributed to consumption of home-made bread prepared from seed wheat treated with mercurial fungicide. The age incidence ranged between 4 and 70 years.Of the various clinical features encountered, neurological symptoms and signs were predominant and included muscular weakness, numbness, unsteady gait, paraesthesia, dysarthria, mental disturbances and, in severe cases, blindness, partial deafness, stupor, coma, and death. Involvement of the cardiovascular, urinary, gastrointestinal and haemopoietic systems, which was commonly encountered in ethylmercury poisoning in the 1960 outbreak in Iraq, was unusual.The severity of symptoms and signs was, broadly speaking, dose-dependent; high exposure led to severe clinical manifestations, but variations existed. Criteria, based on the clinical manifestations, were set for grading the severity of cases. The series included 2 asymptomatic cases, 20 mild, 20 moderate, 14 severe, and 10 very severe. In the latter group 5 patients died from failure of the central nervous system.The severely poisoned patients died irrespective of the medical treatment received. After 2 years of observation, most patients graded as mild or moderate cases lost their symptoms completely. Severe cases improved slowly, although ataxia, diminution of visual field and acuity and paraesthesia were still present. Thus, the previously accepted view that neurological signs were irreversible has been disproved.
 ...
PMID:Intoxication due to alkylmercury-treated seed--1971-72 outbreak in Iraq: clinical aspects. 108 68

Bajiaolian (Dysosma pleianthum), one species in the Mayapple family, has been widely used as a general remedy and for the treatment of snake bite, weakness, condyloma accuminata, lymphadenopathy and tumours in China for thousands of years. However, the textbooks of traditional Chinese medicine mention little about the toxicity of Bajiaolian. Within 1 year, the authors saw five people who manifested nausea, vomiting, diarrhoea, abdominal pain, thrombocytopenia, leucopenia, abnormal liver function tests, sensory ataxia, altered consciousness and persistant peripheral tingling or numbness after drinking infusions made with Bajiaolian. The herb was recommended by either traditional Chinese medical doctors or herbal pharmacies for postpartum recovery and treatment of a neck mass, hepatoma, lumbago and dysmenorrhoea. Podophyllotoxin is one of the main ingredients of the Bajiaolian root. The clinical manifestations observed in our patients were consistent with podophyllum intoxication. Podophyllotoxin intoxication usually results from the accidental ingestion or topical application of podophyllum resin. However, these cases of Bajiaolian intoxication were iatrogenic and results from 'therapeutic doses' of Bajiaolian cited in the textbooks of traditional Chinese medicine.
 ...
PMID:Podophyllotoxin intoxication: toxic effect of Bajiaolian in herbal therapeutics. 136 Nov 36

We report a case of chronic demyelinating polyneuropathy accompanying benign IgM monoclonal gammopathy treated with plasmapheresis, which brought the improvement of the neurological signs and conduction velocities of peripheral nerves. A 60-year-old man developed numbness in the hands and legs and unstable gait. These symptoms became worse slowly. Three years after the onset, he was admitted to Matsuyama Red Cross Hospital because mild clumsiness in the hands was added. The neurological examinations revealed marked loss of both superficial and deep sensations of the glove-stocking type, mild weakness of distal muscles and hyporeflexia in the upper and lower extremities, and mild sensory ataxia. On the laboratory examinations, the serum showed a marked increase of IgM with a monoclonal IgM of lambda light chain. The cytological examination of the bone marrow showed no evidence of malignancy. Marked decrease of nerve conduction velocities was noted in the electrophysiological examinations of the peripheral nerves. Segmental demyelination and widely spaced major dense lines of myelin were observed in the histological examinations of the sural nerve. The immunological examination revealed the antibody activity of IgM against myelin-associated glycoprotein in the patient's serum. He was treated with double-filtration plasmapheresis of 2 liters once a week for 3 months as inpatient. During this treatment, the concentration of IgM in the serum was kept to be much lower than before the treatment, and the sensory disturbances, grasping powers and nerve conduction velocities were mildly improved at the end of the treatment. After discharge, he was treated with monthly plasmapheresis of 2 liters for 3 months.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:[A case of chronic demyelinating polyneuropathy with benign IgM anti-myelin-associated glycoprotein paraproteinenia--transient improvement with weekly plasmapheresis]. 171 May 53

A 41-year-old man was doing well until July 1989, when he noted numbness over soles, followed 4 months later by difficulty in walking. These symptoms were progressively getting worse, and he was admitted to our department on June 12, 1990. General physical examination was unremarkable. Neurologically cranial nerves were intact except old right, traumatic strabismus. Muscle tone and deep tendon reflexes were normal throughout, but bilateral Babinski and Chaddock reflexes were present. Mild weakness of lower extremities were found on muscle testing (4/5). In sensory system, superficial sensory disturbance below T10 was seen, and markedly diminished vibration and position senses of lower extremities were noted. Cerebellar test was intact, although unsteadiness was found on heel-shin test. Romberg sign was definitely positive. His gait was wide-based and ataxic. Laboratory data showed no abnormalities in CBC, chemistry, urinalysis, serological tests and endocrinological examinations. Spinal MRI (Siemens 1.5 Tesla) showed abnormal deposition of epidural fatty tissues compressing spinal cord with flattening of cord from T4 to T8. Spinal ataxia as compressive myelopathy due to epidural lipomatosis was considered and he underwent laminectomy from T4 to T8 with improvement in walking. Epidural lipomatosis is an unusual cause of spinal cord compression, presenting compressive myelopathy, radiculopathy, cauda equina syndrome, intermittent claudication, or back pain. Most of cases were associated with long-term administration of adrenocortical steroid hormone, or underlying diseases, except only 3 cases including ours. This is the first case of spinal epidural lipomatosis presenting progressive gait disturbance due to spinal ataxia.
 ...
PMID:[A case of epidural lipomatosis presenting spinal ataxia]. 206 Feb 46

A 53-year-old man developed spastic ataxia associated with diabetes insipidus. The patient experienced frequent attacks of stiffness and numbness of the four limbs accompanied by difficulty of speech. During an eight years' follow-up a progressive deterioration of the motor function was observed but no extracerebral manifestations were noticed. The association of spastic ataxia is generally considered as diagnostic of histiocytosis X. In the present case, however, multiple sclerosis seems a more likely diagnosis in view of the late onset and the highly characteristic brain stem seizures.
 ...
PMID:Diabetes insipidus in a patient with suspected multiple sclerosis. 238 1

Acute autonomic and sensory neuropathy (AASN), one subtype of acute pandysautonomia, in which dorsal root ganglia and autonomic ganglia are involved is uncommon. Little is so far known on central nervous system involvement in AASN. In the present paper we described a rare case of AASN associated with the central nervous system manifestations such as galactorrhea-amenorrhea syndrome and intractable anorexia. A 30-year-old woman rapidly developed burning pain and numbness in her arms and legs as well as orthostatic syncope. She had severe anorexia and no no menstruation from onset. On physical examination, she was emaciated. There was marked orthostatic hypotension with tachycardia. Skin was dry. Moderate galactorrhea was detected. Neurological examination showed prominent paresthesia and dullness of superficial sensation, predominantly to pinprick and thermal stimuli, segmentally over the neck, occipital scalp, and extremities. Deep sensation was intact. She had no weakness or ataxia. Deep tendon reflexes were almost normal. NCV and SEP were normal, while EEG was abnormal. Sural nerve biopsy demonstrated axonal degeneration with the loss of myelinated, predominantly in small-caliber fibers, and unmyelinated fibers. The levels of HVA and MHPG in CSF were decreased. The autonomic nervous function tests revealed postganglionic dysfunction. alpha-adrenergic system was predominantly impaired, while beta-adrenergic system was relatively preserved. The endocrinological studies demonstrated mild or moderate elevation of PRL basal value and hyper-response of PRL and LH for TRH and LH-RH loading test, which suggested disorder of the hypothalamo-hypophysial system. Cranial MRI showed moderate dilatation of the 3rd ventricle.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:[Acute autonomic and sensory neuropathy associated with galactorrhea-amenorrhea syndrome and intractable anorexia]. 255 96

Only a few cases of carcinomatous neuropathy with tonic pupils have been reported. In the present paper we described a 53-year-old woman with subacute sensory neuropathy who had presented with bilateral tonic pupils. She noticed numbness over the medial aspect of the left thigh in March, 1988, and then developed dysesthesia over the left forearm and hand, mild weakness of left upper and lower extremities and urinary disturbance. Neurological symptoms were subacutely progressive and she was bed-ridden in May. She was admitted to our hospital in June, 1988. On examination, she had body-weight loss of 6 kg during the last six months and general status was otherwise unremarkable. She had anisocoria; the left pupil was larger in daylight than the right, while smaller in dim light. The left pupil scarcely reacted to light, but promptly constricted to near vision. The right pupil constricted normally to light and near vision. An instillation of 0.0625% pilocarpine solution showed supersensitive response of both pupils. An instillation of 1.25% epinephrine solution demonstrated mild dilation of both pupils. Thus, it was conceivable that she had postganglionic ciliary nerve damage characteristic of tonic pupil as well as the lesion of sympathetic nerve innervating pupillary dilator. She had severe sensory ataxia and pseudoathetosis of the hands. Weakness was mild to moderate in extremities. Almost all deep tendon reflexes were absent. All modalities of sensation, particularly on deep sense, were severely involved with sea-level-type distribution below Th7, and over C2 to C3 regions on the left side.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:[Subacute sensory neuropathy manifestated by tonic pupils associated with small cell carcinoma of the lung]. 255 97


1 2 3 4 5 6 7 8 9 10 Next >>