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Query: UMLS:C0004134 (ataxia)
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Organic mercury compounds have greatest affinity to the central nervous system (cerebellar white matter, basal ganglia, occipital and frontal cortex). Anatomical changes observed in the brain are of degenerative type. Similar changes are found also in the myocardium, liver and kidneys. The authors observed for 2 years a family of three persons who contracted poisoning with organic mercury compounds after easting the meat of pigs and hens fed through negligence with wheat destined for sowing, containing methyl-mercurocyanoguanidine. The level of total mercury determined by the method of cold atomic absorption was in these patients four months after poisoning 650,500 and 175 ng/ml of blood (normal value from 1.56 to 18.72 ng/ml). During the observation it has been established that the severity of poisoning was related to the level of mercury found in the organism. Pharmacological treatment (Cuprenil, Thioctacid, vitamin B complex) and rehabilitation brough slight improvement after 2 years. Two patients with total blood mercury level 650 and 500 ng/ml were completely disabled due to encephalopathy with high grade ataxia, dysarthria and concentric narrowing of the field of vision. In the third patients with less severe poisoning (175 ng/ml) pathological manifestations disappeared completely after short treatment.
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PMID:[Encephalopathy after poisoning with organic mercury compounds]. 48 88

Three cases (case 1, female, aged 30; case 2, male, aged 32; case 3, male, aged 34) of benign brainstem encephalopathy with truncal ataxia were reported. Two patients had prodromal symptoms Neurological examination revealed truncal ataxia in all cases. As additional neurological signs, anisocoria, mydriasis, nystagmus, ptosis, transient opsoclonus, and facial palsy were seen. There was neither drowsiness nor myoclonus in the three cases. On laboratory examinations, cold agglutination test revealed significant elevation in two cases. The examination of cerebrospinal fluid showed a moderate rise of proteins in one case, but did not revealed pleocytosis in any of the cases. Magnetic resonance imaging of one patient revealed an area of high intensity in the left pontine tegmentum by T2-weighed imaging. The prognosis for all these cases was good, and the reappearance of neurological signs was not present until now. Our cases were different from brainstem encephalitis (Bickerstaff's encephalitis) because of an absence of disturbed consciousness and no pleocytosis in the cerebrospinal fluid. Our cases were also different from "myoclonus-opsoclonus syndrome" because of an absence of myoclonus. We discussed a possibility of a new clinical syndrome which we call "benign brainstem encephalopathy with truncal ataxia".
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PMID:[Benign brainstem encephalopathy with truncal ataxia--a clinical study of 3 cases]. 128 89

Dextromethorphan-containing cold/cough preparations are frequently prescribed and bought over the counter for use in children. Although generally considered safe, dextromethorphan has been shown to cause CNS side effects, including hyperexcitability, increased muscle tone, and ataxia. Two deaths have been reported with intentional dextromethorphan overdose. A literature review, brief review of pharmacology, and report of two cases of adverse reactions to dextromethorphan-containing preparations are presented.
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PMID:Toxicity with dextromethorphan-containing preparations: a literature review and report of two additional cases. 187 8

A 68-year-old woman presented in wintertime in a cold climate with ataxia and numbness in her legs and was found to be profoundly hypothermic in hospital. No endocrine or neurological cause for hypothermia could be distinguished. Physiological investigation, including a sympathetic release test, exposure to gradually increasing environmental temperatures and prolonged exposure to a high temperature suggested she was at that time regulating her core temperature around a set value which was several degrees lower than normal. Metabolic rate was 42 per cent below the value predicted from standard tables. Further measurements over a one-month period in a warm climate suggested a poikilothermic temperature control mechanism, with a possible risk of environmental hyperthermia. No pathological basis for this disorder has yet been identified, but it is suggested that a small localized hypothalamic vascular event has occurred.
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PMID:Poikilothermia in a 68-year-old female. A risk factor for accidental hypothermia, or hyperthermia. 259 52

The intention of the present study was to characterize patients with central post-stroke pain (CPSP) with regard to type and location of the cerebrovascular lesion (CVL), the characteristics of the pain and the neurological symptoms and signs in addition to the pain. Twenty men and 7 women with a mean age of 67 years and a mean pain duration of 44 months were examined 9-188 (mean 53) months after their stroke. The clinical symptoms and signs and the CT scans indicated that the CVL were located in the lower brain-stem in 8 patients, involved the thalamus in 9 patients and were located lateral and superior to the thalamus in 6 patients. In the remaining 4 patients the location of the CVL could not be determined with certainty. The 3 identified hematomata were all located in the thalamus. The onset of the pain was immediate in 4 patients, within the first post-stroke months in 10 patients and delayed by 1-34 months in the rest. The pain was on the left side in 18 patients. Twenty patients had hemipain. Most patients experienced more than one type of pain. The most common qualities were burning, aching, pricking and lacerating, with some differences in the frequencies according to the location of the CVL. Burning pain was most common, except among the patients with thalamic CVL, in whom lacerating pain was more common. Aching and pricking pain were also frequent. All patients considered the pain to be a great burden and most rated the pain intensity as high on a visual analogue scale. The intensity was increased by external stimuli, the most common being joint movements, cold and light touch. Five patients reported aggravation by emotional stimuli. Besides pain, the only neurological symptom common to all patients was decreased temperature sensibility, as shown by quantitative methods. It is possible that pain sensibility was also abnormal in all. Hypersensitivities to cutaneous stimuli, including evoked dysesthesias were found in 88% of the patients, while the detection thresholds for touch and vibration were abnormal in only 52% and 41%, respectively. Similarly, low figures were found for paresis and ataxia, which were present in 48% and 62%, respectively. It is concluded that only a minority of patients with central pain after stroke have thalamic lesions.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Central post-stroke pain--neurological symptoms and pain characteristics. 291 91

Clinical observations are presented on the sensory effects of lesions of different afferent pathways of the spinal cord, correlated whenever possible with histological evidence of the location and extent of the lesions. They are based on personal cases and on significant cases in the literature, including posterior column section, other causes of damage to the posterior columns, and cases of commissural myelotomy. It is concluded that the traditional view of the effects of lesions of the posterior columns is correct, but that evidence from cases proved by postmortem examination is still needed. When the information normally supplied by the posterior columns is cut off, primary sensibility for light touch and pressure is not lost, but any kind of discrimination is disturbed. There is also a disturbance in knowledge of movement and position, ataxia, and clumsiness in the use of the hands. These defects greatly affect the palpatory examination of objects and, although they may appear slight on routine neurological examination, they can cause severe disturbances in the activities of daily living. For tactile modalities, a lesion of the spinothalamic complex causes minimal or no defects and a lesion of the posterior columns causes only slight defects, whereas a lesion of both pathways gives rise to total loss of tactile and pressure sensibility in the part of the body served by both pathways. This conclusion is based on 2 cases with combined commissural myelotomy and anterolateral cordotomy. The following disturbances of mechanoreception attributed to lesions of the posterior columns are discussed: lability of threshold, persistence of sensation, tactile and postural hallucinations and temporal and spatial disturbances. In man, lesions of the posterior columns cause an increase in pain, tickle, warmth and cold. Cases are presented with and without lesions of the posterolateral columns in conjunction with lesions of one or both anterolateral columns. As these lesions did not affect sensation and as there was no difference in the sensory state following anterolateral cordotomies with or without involvement of the posterolateral column, it is concluded that lesions of this column have no effect on sensation. Cases with lesions of the anterior two-thirds of the cord are also presented to illustrate the sensory state with only the posterior third of the cord intact. In these cases, tactile and pressure sensibility and knowledge of movement and position are normal.
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PMID:Sensory effects in man of lesions of the posterior columns and of some other afferent pathways. 309 88

A case of an anteriorly located meningioma in the region of the foramen magnum, successfully treated by a transoral approach, is reported. A 60-year-old woman was admitted to the author's department on January 19th, 1983. Six years before admission, she began to experience the gradual cold dysesthesia in the both palms, and then developed gait disturbance, clumsiness of the right hand and a stiffneck. She was found to have atrophied hands, mild spastic tetraparesis, ataxia and hypalgesia under the bilateral C2 spinal levels. A CT scan disclosed a patchy hyperdense lesion, which was enhanced homogenously with contrast medium, occupying almost all the intradural extramedullary space at the foramen magnum. The transoral approach was employed following preliminary tracheostomy. The tumor, extending about one centimeter superiorly to the basion and inferiorly to the upper border of C2 body, was removed completely, and then the dural defect was covered with a piece of fascia and gelform. An anterior occipitocervical fusion was performed extending to C2 using a block of an antogenous iliac bone. Post-operatively immobilization was secured by a Halo-Vest. But unexpectedly, CSF leakage was disclosed several days after the surgery without neurological deterioration. On the 17th day following first surgery, a re-operation was performed. The dural defect was successfully repaired with a piece of fascia by interrupted sutures in an illustrated fashion (Fig. 5). A piece of muscle was then placed in the epidural space created by bone removal. A fusion of the atlanto-axial joint not extending to the basiocciput was employed.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Transoral anterior approach to foramen magnum meningioma--a case report and review]. 388 68

Chronic states of methylmercurialism were induced in squirrel monkey subjects. Principal neurological signs included ataxia, abnormal gait, incoordination and amaurosis. Although slight to moderate vacuolization occurred in supporting cell layers of the cristae and maculae, receptor cell function was essentially normal. Except for a lowered cold threshold, bithermal caloric-induced nystagmus was not significantly different from control values. Pre and postrotatory (Barany chair) tests revealed a reduction only in frequency related variables. The development of spontaneous and positional nystagmus (sometimes with eyes open) coupled with the behavioral signs and the evidence of normal receptor response suggested cerebellar dysfunction. Severe pathologic changes were present in the cerebral cortex, but no lesions were found in the cerebellar cortex. Substantial neuronal degeneration and gliosis, however, were observed in several subcortical nuclei, including cerebellar and vestibular nuclei.
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PMID:Horizontal nystagmus in methylmercury poisoned squirrel monkeys. 613 46

We have measured in leukocytes the following lysosomal enzymes in 11 Friedreich disease cases, 11 "atypical" recessive ataxias, 13 neurological controls and 16 normal controls: hexosaminidase A and B; beta-galactosidase and neuraminidase (labile and cold stable, or A and B). The lysosomal enzyme deficiencies known to produce certain forms of spinocerebellar degeneration were not present in Friedreich's disease or the Charlevoix-Saguenay syndrome. The very small scale survey of "atypical" recessive ataxias revealed 3 cases of severe deficiencies in hexosaminidase activity. Two adult brothers presenting with the clinical phenotype of Kugelberg-Welander disease (one also with ataxia), were shown to have a severe deficiency of both HEX A and HEX B activity (Sandhoff biochemical pattern). This is the first such report. A further adult female patient, unrelated to the others, had a severe isolated deficiency of HEX B and presented with a very slowly progressive and mild ataxia with severe internal strabismus. These patients and their families are being studied clinically and biochemically in greater detail and will be reported elsewhere. However these preliminary findings justify screening for such lysosomal defects in all cases of "atypical" recessive ataxia.
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PMID:Lysosomal enzymes in ataxia: discovery of two new cases of late onset hexosaminidase A and B deficiency (adult Sandhoff disease) in French Canadians. 623 79

Magnesium deficiency may complicate many diseases. The causes include the following: inadequate intake during starvation or increased requirement during early childhood, pregnancy, or lactation; excessive losses of magnesium as a result of malabsorption from the gastrointestinal tract or from the kidneys during use of diuretics; and to a combination of the two, as in alcoholism. Most often the etiological factors have been operative for a month or more. Acute hypomagnesemia can occur without previous Mg deficiency after epinephrine, cold stress and stress of serious injury or extensive surgery. The clinical manifestations depend on the age of the patient and may begin insidiously or with dramatic suddenness, or there may be no overt symptoms or signs. The manifestations can be divided into the following categories: totally non-specific symptoms and signs ascribable to the primary disease; neuromuscular hyperactivity including tremor, myoclonic jerks, convulsions, Chvostek sign, Trousseau sign (rarely), spontaneous carpopedal spasm (rarely), ataxia, nystagmus and dysphagia; psychiatric disturbances from apathy and coma to some of all facets of delirium; cardiac arrhythmias including ventricular fibrillation and sudden death; hypocalcemia which is responsive only to Mg therapy; and hypokalemia which is not easily nor completely corrected without Mg therapy. The diversity of etiologies and the multiplicity of manifestations result in confusion and controversy. The documentation of normal renal function is absolutely necessary for maximum doses. The order of magnitude of dose is 1.0 meq Mg/kg on day 1, and 0.3 to 0.5 mEq/kg per day for 3 to 5 days. In emergencies such as convulsions or ventricular arrhythmias, a bolus injection of 1.0 gm (8.1 meq) of MgSO4 is indicated. Therapy of Mg deficiency in the presence of renal insufficiency requires smaller doses and frequent monitoring. Complete repletion occurs slowly.
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PMID:Magnesium deficiency. Etiology and clinical spectrum. 702 Mar 47


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