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Query: UMLS:C0004134 (ataxia)
 15,886 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We present two siblings with hereditary cortical cerebellar atrophy (CCA), who showed peculiar clinical features. Their unaffected parents are cousins. The mode of inheritance in this family was autosomal recessive. Both patients developed involuntary movement and ataxia during the fourth decade. The proband (patient 1) was the elder sister. She developed choreoathetoid involuntary movement and cerebellar ataxia at the age of 32. At the age of 39, she showed mental deterioration and marked gait disturbance due to severe ataxia and amyotrophy. At the age of 40, she took medication for hypertension. At the age of 42, she was bedridden and had generalized convulsions and dysautonomia. Involuntary movement continued until her death at age 44. She had amenorrhea since the age of 25 years. Neuropathological findings. The brain weighed 1,010 g. We found marked degeneration in the cerebellar cortex including the molecular, Purkinje cells, and granular cell layers, and in the inferior olivary nuclei. In the basal ganglia, the putamen and caudate nuclei were moderately affected, but the substantia nigra and globus pallidus were spared. The cerebral cortex was spared, but the cerebral white matter showed diffuse myelin pallor without fibrillary gliosis. In the pons, the volume of the tegmentum was moderately decreased, but the base was spared. The spinal cord was normal. The findings of the patient differed from those of the case originally reported by Gordon Holmes in 1907. Holmes autopsied a case showing severe degeneration in both the cerebellar cortex including all three layers and the inferior olivary nucleus as in our patient. However, the striatum of his case spared and the patient did not develop involuntary movement as did other patients. The patients presented here should be distinguished from Holmes' original case clinicopathologically.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Autosomal recessive hereditary cortical cerebellar atrophy with striatal degeneration--two siblings showing choreoathetoid movement, ataxia, dementia, and amenorrhea]. 806 39

Radiological and clinical analysis was performed in 5 patients with posterior fossa dural arteriovenous fistulas (DAVFs) with isolated sinus segment due to sinus thrombosis, and their patho physiological, diagnostic, and therapeutic concerns were discussed. Patients ranged in age from 36 to 73 years old with a mean of 57 years and included 2 males and 3 females. Two patients presented with ataxia, one with ataxia and bruit, one with atypical facial pain, and one with parietal dysfunction. One patient had a history of hemorrhagic event. DAVFs were located at transverse-sigmoid sinus (3 cases), superior petrosal sinus (1 case), and straight sinus (1 case). They were fed by many branches of external carotid artery including middle meningeal artery, ascending pharyngeal artery, posterior auricular artery, occipital artery, meningeal branches of vertebral artery and posterior cerebral artery, and meningohypophyseal trunk of internal carotid artery. Shunt flow drained into contralateral transverse-sigmoid or supratentorial sinuses via the isolated venous segment through markedly dilated cortical and/or deep venous systems, which caused altered normal venous drainage pattern and venous hypertension. Transarterial embolization in multiple stages (mean 3.4) using n-butyl cyanoacrylate (NBCA) could alleviate symptoms in all cases. DAVFs were almost totally obliterated in 3 patients. Further embolization in one case, and surgical excision in one case were planed because of some residual dilated cortical venous drainage. Posterior fossa DAVFs with isolated sinus segment accompany markedly dilated cortical and/or deep venous systems. They could cause cerebellar, brainstem, or cranial nerve dysfunctions, and sometimes present distant supratentorial symptoms or hydrocephalus due to abnormal venous drainage and venous hypertension.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Posterior fossa dural arteriovenous fistula with isolated sinus segment]. 821 93

The frequency of cerebellar infarctions over two and a half years was 2.7% of the 1300 hospitalized patients over that period. Sixteen patients with cerebellar infarctions were studied by using clinical manifestations and magnetic resonance imaging (MRI). Ages ranged from 41-87 (mean 63.5) years; 13 were men and 3, women. Risk factors included: hypertension (50%), diabetes (44%), prior stroke (44%), cardiac disease (38%), and hyperlipidemia (19%). Common symptoms and signs were dizziness/vertigo (75%), unsteadiness (69%), dysarthria (69%), and nausea/vomiting (50%). Infarcts mainly involved the posterior inferior cerebellar artery (PICA) territory and tended to be associated with brainstem infarcts in 14 of the 16 patients. Most cerebellar infarctions had a benign course, especially the small ones. No mortality was noted in this series. The short-term outcome of the cerebellar infarctions seemed to depend on the size of the infarcts and the sites of the artery occlusion. It was concluded that diagnosis of cerebellar infarctions requires a high index of clinical suspicion, especially when patients present with a sudden onset of ataxia, dizziness/vertigo, nausea/vomiting and dysarthria; and that MRI is a useful tool for the detailed study of cerebellar infarctions and can elucidate associated brainstem infarcts.
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PMID:A clinical and MRI study of cerebellar infarctions. 829 26

Spontaneous midbrain hemorrhage is a unique brain-stem vascular lesion. Sixty-six cases have been reported in the literature, to which we add seven. Of the total of 73 patients reviewed, most presented acutely (66%), but subacute (22%) and chronic onsets (12%) also occurred. Neuro-ophthalmologic abnormalities were found in 88% of patients. Some alteration of consciousness and headache occurred in most patients. Hemiparesis, hemisensory loss, and ataxia were less frequent manifestations. Vascular malformations were suspected or proved in 37% of patients, hypertension was present in 21%, and a bleeding diathesis was noted in 5%. No underlying cause was apparent in 37%. Most patients improved with supportive care only. At follow-up, 23% of patients had no neurologic deficits. In 51% of patients, minor neurologic deficits persisted, related chiefly to cranial nerves III and IV. Moderate deficits persisted in 16% of patients, and 5% of patients died.
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PMID:Spontaneous midbrain hemorrhage: report of seven new cases. 845 23

Twenty-five patients with various types of gait disorders of multi-infarct dementia (MID) were reported. The types of gait disorders consisted of lower body parkinsonism (LBP) plus ataxia (6 patients), LBP plus apraxia (5 patients), and a combination of LBP plus ataxia and apraxia (14 patients). Hypertension occurred in 23 (92%) of the 25 patients. Nevertheless, individual stroke risk factors and the locations of infarcts were not significantly different between the subgroups. Ventriculomegaly and "leuko-araiosis" as demonstrated by computed tomography occurred in more than 80% of patients in each subgroup. Atrophy of the superior vermis was seen in 16 (80%) of 20 patients with ataxia as compared to 2 (40%) of the 5 patients without ataxia (p < 0.005). These data suggest that LBP and apraxia of MID were probably determined by the presence of ventriculomegaly or leuko-araiosis or both, and the presence of ataxic component of gait disorder most probably indicates the presence of vermian atrophy.
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PMID:Gait disorders of multi-infarct dementia. CT and clinical correlation. 847 97

We report a patient who developed an acute, reversible, generalized choreiform disorder from lithium (Li) intoxication. This medication was prescribed for manic-depressive disorder, and serum levels became elevated after the addition of a diuretic for the treatment of hypertension. There were no other apparent causes for the movement disorder, and it was associated with other known features of Li intoxication, including ataxia and encephalopathy. There was a delay between the initial symptoms of Li intoxication and the onset of chorea. The chorea improved as serum Li levels diminished, with some lag time. This represents the eleventh case report of Li-induced chorea, but only the sixth in a patient without concomitant neuroleptic therapy, and the first presented with videotape confirmation. A review of these other cases is included, and possible mechanisms are discussed.
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PMID:Chorea caused by lithium intoxication: a case report and literature review. 891 4

We report a 91-year-old man who had a stroke and died of renal failure. He had been treated for hypertension since 20 years before the onset of the present illness. In addition, he was operated on a gastric cancer 17 years previously. Otherwise he was doing well until May 29, 1991 (when he was 87-year-old) when he had sudden onset of dysarthria and right facial weakness. He was admitted to our hospital. On admission, general physical examination was unremarkable, and neurologic examination revealed a mentally sound man with slight dysarthria, right facial weakness, orolingual dyskinesia, and dysequilibrium in which he showed difficulty in tandem gait; however, no cerebellar ataxia was noted. A cranial CT scan revealed leukoaraiosis with multiple low density areas in the cerebral white matter. His BUN was 37 mg/dl and Cr 2.2 mg/dl. His neurologic symptoms cleared within the next few weeks and he was discharged with ticlopidine 100 mg q.d.. He had been doing well after the discharge except for gradual worsening of his renal function; his BUN was 65 mg/dl and Cr 3.27 mg/dl in April of 1994. On March 10, 1995, he fell down and hit his back; he became unable to walk because of pain, and he was admitted again on March 16, 1995. On admission, his blood pressure was 170/80 mmHg. There was an 1 + pitting pretibial edema; otherwise general physical examination was unremarkable. Neurologic examination revealed an alert and oriented man, however, Hasegawa's dementia scale was 23/30. Higher cerebral functions as well as cranial nerves were intact. He showed some unsteadiness of gait, however, no motor weakness or ataxia was noted. Deep tendon reflexes were diminished, but Chaddock sign was positive bilaterally. Vibration was diminished in the feet, however, pain and touch sensations were intact. Laboratory examination revealed a compression fracture of the twelfth thoracic vertebra. Blood count and chemistries were as follows; Hb 7.6 g/dl, Hct 23.3%, TP 6.0 g/dl, Alb 3.6 g/dl, BUN 87 mg/dl, Cr 4.53 mg/dl, T-Chol 174 mg/dl, HDL-Chol 49 mg/dl, Glu 156 mg/dl, Na 142 mEq/L, K 5.4 mEq/L, Cl 115 mEq/L. A urine specimen contained 1 + protein and 1 + glucose, and the sediments contained hyaline casts. A cranial CT scan was essentially same as that taken four years ago. His hospital course was complicated with pneumonia, congestive heart failure, and progressive renal failure. He was treated with intravenous fluid, chemotherapy, and other supportive measures, however, he expired from respiratory failure on April 30, 1995. He was discussed in a neurologic CPC, and the chief discussant arrived at the conclusion that the patient had Binswanger's disease in the brain, benign nephrosclerosis from arteriolosclerosis due to hypertension, congestive heart failure, and pneumonia. Opinions were divided regarding the question as to whether or not this patient had Binswanger's disease. Although his cranial CT scan revealed leukoaraiosis, his dementia and gait disturbance was only mild until his fall on March, 1995. Clinical features did not conform to those of Binswanger's disease. Postmortem examination of the right hemisphere revealed wide spread atherosclerosis and arteriolosclerosis. The kidney showed benign nephrosclerosis due to arteriolosclerosis. Sclerotic changes were also seen in the coronary arteries and the left middle cerebral artery with 70% stenosis. Myelin stain showed diffuse myelin pallor of the cerebral white matters with scattered small infarcts. Arterioles in the white matter showed arteriolosclerosis. Small infarcts were also seen in the putamen and in the thalamus. This patient appeared to have had circulatory disturbance of the white matter which is the basic abnormality causing Binswanger's disease. However, white matter changes in this patient were not quite severe enough to make a pathologic diagnosis of Binswanger's disease.
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PMID:[A 91-year-old man with a stroke, hypertension, and renal failure]. 899 Apr 84

The clinical and cardiorespiratory effects of premedication with acepromazine, butorphanol or diazepam in addition to romifidine before induction of anaesthesia with ketamine were studied in 6 horses on 4 random occasions. Administration of romifidine alone or in combination with butorphanol resulted in an increase in arterial blood pressure, accompanied by a significant decrease in heart rate with second-degree atrio-ventricular heart block. Induction of anaesthesia with ketamine returned the heart rate to baseline value, but the arterial blood pressure was significantly increased compared to baseline. Including acepromazine in the premedication prevented the hypertension and bradycardia induced by romifidine. The respiratory rate was slightly decreased after premedication in all groups, but returned to the baseline value after induction of anaesthesia. Mild hypercapnia and significant hypoxaemia were observed during sedation and anaesthesia, reflecting an impairment of pulmonary function. Premedication with acepromazine before sedation with romifidine resulted in a fast induction and good anaesthesia. Inclusion of butorphanol in the premedication resulted in individual variation in the quality of induction and anaesthesia. Addition of diazepam to the sedation with romifidine resulted in good muscle relaxation with a smooth induction and maintenance of anaesthesia and an increased time before the horses responded to noxious stimuli, compared with romifidine and ketamine anaesthesia. All horses reached a standing position at the first attempt, but horses premedicated with diazepam in combination with romifidine showed mild ataxia after recovery.
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PMID:Effects of additional premedication on romifidine and ketamine anaesthesia in horses. 899 76

Acute infarcts of the anterior inferior cerebellar artery (AICA) are unusual. We report 15 cases of AICA infarcts and their correlation with the topography of the lesion by brain MRI. During 2 years we prospectively identified 7 cases of AICA infarcts among 770 acute strokes (0.9% of the acute strokes seen in our department). We studied these cases and also another 8 that we found retrospectively. Most patients (8/15) had a unilateral affectation of both middle cerebellar peduncle (MCP) and inferior lateral pontine area (ILP), in these cases the main symptoms were vertigo, ataxia, peripheral facial palsy and hypoacusia. Two other patients had isolated MCP infarcts and were characterized by peripheral vertigo and ataxia, without hypoacusia or facial palsy. Another 2 patients had isolated ILP territory infarct characterized by vertigo, left peripheral facial palsy without hypoacusia and mild or no ataxia. One patient had a Gasperini syndrome. Finally 3 patients had bilateral AICA infarcts due to basilar thrombosis. The etiology was atherosclerosis in 9 patients, lacunar due to hypertension in 1, cardiac embolism in 1, migraine in 1 and unknown in 3. Among the 15 patients only 2 died, both with AICA plus infarcts. In the remaining patients a follow-up during a mean of 31 months (3 months to 12 years) showed no recurrences.
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PMID:The anterior inferior cerebellar artery infarcts: a clinical-magnetic resonance imaging study. 957 36

We report a rare case of a second primary brain tumor following esophageal cancer. A 56-year old Japanese man underwent a transhiatal esophagectomy for esophageal carcinoma. Two years after the operation, signs of increased intracranial hypertension including headache, nausea and left limb ataxia were observed. The tumor was totally removed by a suboccipital craniectomy, and the histological diagnosis was hemangioblastoma. The post-operative course was uneventful and the patient is still alive without any signs of recurrence. It is important to make a precise strategy for the second primary tumor.
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PMID:Esophageal cancer and second primary brain tumor. 968 23


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