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

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

A patient with a 2-year history of progressive ataxia and tingling in the right hand had prominent facial myokymia. Magnetic resonance imaging revealed syringomyelia and syringobulbia. After successful syringosubarachnoid shunting, the patient died of massive pulmonary embolism. Postmortem examination revealed a syrinx involving the spinal cord and lower half of the medulla; neither the facial nucleus nor facial nerve fibers were directly involved. We hypothesize that interruption of aberrant corticobulbar fibers in the medulla produced disinhibition of a rhythmic neural generator in the facial nucleus.
 ...
PMID:Facial myokymia in syringobulbia. 218 81

Two incidents of toxin-type food poisoning in N.E. Scotland associated with the consumption of red whelks (Neptunea antiqua) are described. Four patients developed symptoms within 1 h of consuming whole whelks. These included visual disturbances--double vision and difficulty in focusing--tingling of the fingers, prostration and in one subject nausea, vomiting, diarrhoea and ataxia. In all cases recovery was complete in 24 h. Using a newly developed analytical technique the concentration of the causative toxin, tetramine, in the salivary glands of the whelks consumed was estimated at 0.07%, equivalent to a content of 3.75 mg/100 g of the shellfish.
 ...
PMID:Food poisoning due to the consumption of red whelks (Neptunea antiqua). 318 22

We report a case of very slowly progressive, high-cervical spondylotic myelopathy with symmetrical deep sensory deficits in the palms. A 76-year-old man began to feel tingling sensation in the second fingers of the bilateral hands 30 years prior to admission. The abnormal sensation spread from the first to the third fingers, and subsequently all over the palms. He noticed intermittent sharp pain in the dermatomes of C4 and 5 bilaterally from his late sixties, and later he developed clumsiness of fine finger movements. In recent years he experienced stiffness in the thighs while walking. On neurological examination, there was a mild sensory deficits in light touch over the bilateral palms, while perception of temperature and pain was normal. Vibration sense was severely and position sense mildly impaired. Discriminative sensation, including graphesthesia, stereognosis, two-point discrimination and texture recognition, was severely impaired over the bilateral palms. On the other hand, all modalities of sensation were normal in the lower limbs. Gross motor dysfunction, such as weakness of limbs, amyotrophy or gait disturbance, was not present. He did not show limb ataxia, but the dexterity of his fingers was severely impaired. Deep tendon reflexes were mildly increased except for the Achilles tendons that were hyporeactive. Plantar responses were flexor bilaterally. Nerve conduction study revealed giant F waves and H reflexes by stimulations of the median and ulnar nerves bilaterally.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:[A case of very slowly progressive, high-cervical spondylotic myelopathy presenting with symmetric deep sensory deficits in the palms]. 754 39

We report a 55-year-old man with papilledema and multiple cranial nerve palsies. He was well until 52 years of age when there was an onset of progressive difficulty in initiating urination; he visited the urology service of our hospital where a diagnosis of prostate cancer was made; the cancer was invading the bladder and was metastasizing to lymph nodes and bones. He was treated with oochiectomy and estrogen preparations with some improvement in his symptoms. Two years later, he developed difficulty in urination again, and transurethral resection of the tumor was performed in 1991. In December 1991, he noted tingling and numb sensation in his left face, which had become progressive worse within the next one month, and he developed blepharoptosis and deafness all on the left side. He was admitted to the urology service on February 4, 1992, and a neurological consultation was asked. On physical examination, general findings were unremarkable, except for lymph node enlargements of about 0.5 to 1.0 mm in size in cervical and inguinal regions. On neurologic examination, he was alert with normal mental activities; higher cerebral functions were intact. He had normal vision and visual fields, however, papilledema was present bilaterally; pupils and light reactions were normal. Extraocular muscles were intact on the right side, however, moderate restriction was noted in the left eye in that all the extraocular muscles except for the medial rectus were weak; blepharoptosis was noted on the left; no nystagmus was present. The sensation was diminished in the left face, and left facial paresis of the peripheral type was also noted; the taste sensation was also diminished in the left anterior two thirds of the tongue. He had sensorineural deafness on the left side. The other cranial nerves appeared intact. He walked normally; no weakness or muscle atrophy was noted; muscle tone was normal and no ataxia was observed. Deep reflexes were normally elicited and symmetric; the plantar response was flexor. No meningeal signs were present. Laboratory examination revealed following abnormalities: Hb 7.1 g/dl, platelet 47,000/cmm, WBC3,800/cmm, LDH 950IU/l, PAP232ng/ml (normal less than 1.6), PA2.631ng/ml (normal less than 7.4); a small amount of effusion was noted in the left pleural cavity; cytological examination of the fluid was class V. A cranial CT scan as well as MRI were entirely normal, as was the spinal tap. He was treated with glycerol, however, there was progressive increase in the pleural effusion, and he developed dyspnea; moist rale had become audible in the end of February.(ABSTRACT TRUNCATED AT 400 WORDS)
 ...
PMID:[A 55-year-old man with prostate cancer, papilledema, and multiple cranial nerve palsies]. 794 37

A patient with ataxic hemiparesis and ipsilateral cheiro-oral dysesthesia (tingling) due to a single lacunar infarct at the border between the posterior limb of the internal capsule and the corona radiata is described. A 47-year-old hypertensive man was admitted because of weakness of the right upper and lower limbs with ipsilateral peri-oral and palmar dysesthesia, which developed during bathing in the morning. Upon admission, neurological examination revealed right hemiparesis with ipsilateral limb ataxia and ipsilateral peri-oral and palmar dysesthesia. Based on neurological finding, he was diagnosed as having ataxic hemiparesis with ipsilateral cheiro-oral syndrome. Brain computed tomography and magnetic resonance imaging demonstrated a small lesion at the border between the posterior limb of the left internal capsule and the corona radiata, which was enhanced with gadolinium-DTPA administration. It has been reported that the disturbance of the cortico-pontine tract causes ataxic hemiparesis and that of the thalamo-cortical projection causes cheiro-oral syndrome. These findings of the present case, therefore, suggest that both of these pathways run closely together at the border between the posterior limb of the internal capsule and the corona radiata.
 ...
PMID:[Ataxic hemiparesis with ipsilateral cheiro-oral syndrome due to a single lacunar infarct at the border between the posterior limb of the internal capsule and the corona radiata]. 819 70

We report a 32-year-old man who developed cerebellar ataxia and a posterior fossa mass 12 years after the radiation therapy for a cerebellar arteriovenous malformation (AVM). The patient was well until 19 years of the age when he had an acute onset of vertigo and vomiting. A spinal tap was performed and the CSF was bloody. He was admitted to another hospital where an arteriovenous malformation was found in the cerebellum by angiography. Four years after the onset, he developed tingling sensation in the distribution of the second division of the right trigeminal nerve. He was admitted to the neurosurgery service of our hospital where the cerebellar AVM was confirmed. He was transferred to University of California where Bragg peak stereotaxic radiotherapy was successfully performed; this utilizes high energy alpha-ray produced by a cyclotron. Three years after the radiotherapy, marked reduction in the size of the AVM was confirmed by angiography. Twelve years after the onset of his initial symptom, he noted unsteadiness of gait. He was readmitted to our neurosurgery service where obstructive hydrocephalus was found. He was treated by ventriculoperitoneal shunting and placement of a Ommaya reservoir. After these therapy, he noted marked improvement in his gait and ataxia. However, in 1993, his unsteadiness of gait recurred, and he was again admitted to our neurosurgery service on June 20, 1993. On admission, T1-weighted MRI revealed a slightly low signal intensity mass lesion in the right cerebellar hemisphere compressing the brain stem; a spotty high signal intensity lesion and another small low intensity lesion were seen within the mass. Vertebro-basilar angiograms revealed upward displacement of the superior cerebellar arteries. No arteriovenous nidus was visualized. On July, 3rd, the cyst was surgically drained and the Ommaya reservoir was removed. Post-operative course was uneventful, however, he developed head tremor after the surgery. Neurologic examination on July 20, 1993 revealed an alert and well oriented man in no acute distress. General physical examination was unremarkable. Neurologic examination revealed no dementia; higher cerebral functions appeared intact. The optic discs were flat, and visual fields were intact. Ocular movements were full but convergence was restricted. Horizontal gaze nystagmus was noted more in the right lateral gaze. Pupils were intact. Facial sensation and facial muscles were intact. Hearing was normal. His voice was of nasal quality. Pharyngeal reflex was diminished. The tongue showed deviation to the left without atrophy. Head tremor at 5 c/s was noted. He was able to stand with support but was unable to walk. No muscle atrophy or weakness was noted. The finger-to-nose and the heel-to-knee tests showed dysmetria and decomposition more on the right. Rapid alternating movements were ataxic on the right. Muscle tone was diminished on the right. Muscle stretch reflexes were normally elicited and were symmetric. The plantar response was flexor bilaterally. Sensation was intact. On July 21, a posterior fossa exploration was performed. After the surgery, he was treated with 30 mg/day of alotinolol which showed no effect on his head tremor. He was then treated with gradually increasing doses of clonazepam; when he received 8 mg/day of clonazepam, his tremor showed marked improvement. He was discussed in a neurologic CPC on the nature of the posterior fossa lesion and his tremor. Opinions were divided between delayed radiation necrosis and a radiation-induced brain tumor. The chief discussant arrived at the conclusion that the patient had delayed radiation necrosis compressing the brain stem and cerebellar hemispheres. Regarding the nature of his tremor, he thought that his head tremor was of cerebellar type of postural tremor. Histologic examination of the biopsied specimen revealed accumulation of relatively fresh blood constituents in the deep area of the cerebellum forming a mass. Most of the
 ...
PMID:[A 32-year-old man who developed a posterior fossa mass 12 years after the radiation therapy for cerebellar arteriovenous malformation]. 867 25

We report a 67-year-old hypertensive right-handed woman who developed severe pain and dystonia in her left upper and lower extremity after a thalamic infarction. She was well until 9 months prior to the present admission to our hospital, when she had an acute onset of left hemiparesis which turned out to have been caused by a thalamic infarct. Her hemiparesis showed nearly complete recovery during the next four months. She noted an onset of severe spontaneous pain and difficulty in using her left hand four months prior to the present admission. Neurologic examination on admission revealed an alert and well oriented Japanese woman. Cranial nerves were intact. Although she did not have weakness, her left hand showed thalamic posture, and upon standing, she showed a dystonic posture in which her left forearm took pronation and flexion at the elbow joint and her left lower extremity took extension in the knee joint and planter flexion in the ankle joint. Her dystonic posture increased during walking and disappeared in the supine position. She complained of severe spontaneous pain and tingling sensation in her left extremities. Position sense was diminished in her left leg. However other sensations were intact. She had slight ataxia on the left side. Deep tendon reflexes were symmetric, but the planter response was extensor on the left side. MRI revealed a small lacunar infarct involving the right posterolateral thalamic region. EMG with surface electrodes revealed non-reciprocal tonic discharges in the left biceps brachii and forearm flexor and extensor muscles. She responded poorly to various medications. Only trihexyphenidyl showed partial alleviation of her pain and dystonic posture. We thought her pain might be caused by dystonic contraction of the skeletal muscles, at least in part. We injected 25 IU of botulinus toxin as a total dose into her biceps brachii, triceps brachii, and wrist flexor muscles. A few days after the injection, her dystonic posture began to show marked improvement; as her dystonia improved, her pain also showed marked improvement. This patient appeared to represent a case of post-hemiplegic dystonia. Her pain was initially thought to be the thalamic pain. However, as her pain disappeared with improvement of her dystonia, her pain is most likely to have been caused by the dystonic muscle contraction. Botulinus toxin treatment appears to be useful for post-hemiplegic painful dystonia.
 ...
PMID:[A case of post-hemiplegic painful dystonia following thalamic infarction with good response to botulinus toxin]. 949 Aug 97

A case report is presented of an electrophysiologic recovery from vitamin E-deficient neuropathy after treatment with water-soluble vitamin E in a patient with chronic hepatobiliary disease. The patient was a 64-year-old man who had experienced progressive difficulty in ambulation, with ataxia, over the previous 3 years. The symptoms were associated with pain, tingling sensation in the extremities, and reduced fine motor activity. The patient had chronic hepatobiliary disease, with recurrent cholangitis and external drainage of bile acid through a T-tube for more than 20 years. Vitamin E level was barely detectable (<0.5 mg/L). Sensory conduction was absent in both sural nerves. Other sensory and motor conduction studies in the upper and lower extremities showed decreased amplitude. The patient was treated with water-soluble vitamin E. After 4 months of therapy, his ambulation function improved, but pain and tingling sensation in both hands remained. Sensory nerve action potentials appeared in both sural nerves, and amplitudes of other sensory nerves were increased. In a second follow-up study after 9 months, all of the evaluated parameters in the nerve conduction studies, as well as the vitamin E level, were normal. The authors conclude that vitamin E-deficient neuropathy is reversible and electrophysiologic recovery can occur with water-soluble vitamin E therapy.
 ...
PMID:Electrophysiologic recovery after vitamin E-deficient neuropathy. 1045 75


1 2 3 Next >>