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

In spontaneous cerebellar hemorrhage emergency surgical intervention is often life-saving. Clinical features and the operative results of hypertensive cerebellar hemorrhage (18 cases) were compared with those of hemorrhage caused by small angiomas (7 cases). Hypertensive hemorrhage occured most frequently in the seventh decades. Two thirds of the patients developed brainstem compression syndrome within a week from onset. One third remained awake or drowsy throughout their clinical course. Surgical removal of a hematoma was carried out in 13 patients with four deaths. Of note, two comatose patients regained consciousness after surgery, and were discharged with residual ataxia. Rupture of a small angioma occurred in younger patients. Their clinical course was sub-acute or chronic associated with focal cerebellar dysfunction. All seven surgically treated patients subsequently regained independent function. CT findings have been found helpful not only for diagnosis but also in defining appropriate therapy. Hematomas larger than 3 cm in diameter produced signs of rapidly progressing compression of the brainstem. Thereby, regardless of the cause of bleeding, emergency removal of a clot is indicated even in awake patients. Hematomas of 2 to 3 cm produced brainstem compression or prolonged cerebellar dysfunction, and occasionally require surgical decompression. Hematomas smaller than 2 cm can be managed conservatively, since they were absorbed spontaneously in three weeks without residual functional disturbances. However, in case of a young patient exploration should be performed for a probable "cryptic" angioma.
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PMID:[Diagnosis and treatment of cerebellar hemorrhage: comparison of hypertensive hemorrhage with hemorrhage caused by small angiomas, and CT findings (author's transl)]. 49 56

We report a 54-year-old woman who revealed right hemiparesis and ataxia in her right extremities due to a lesion which was suggested to be cavernous hemangioma located in the left side of pons in 1971, and showed remarkable crossed cerebellar atrophy (CCA) by computed tomography and magnetic resonance imaging in 1985 and 1989. Angiography showed no abnormality but made iatrogenic embolism in left thalamus which developed severe pain in her right side. Single-photon-emission-computed-tomography (SPECT) with I123-IMP in 1989 showed reduction of right cerebellar hemispheric blood flow (crossed cerebellar diaschisis: CCD) and left cerebral hemispheric blood flow. Our case was thought to have revealed persisting CCD. Although there are many reports about CCD and CCA following cerebral damage, CCD and CCA due to brain stem lesion has not been described yet. This might be explained by the persisting functional depression of cerebro-ponto-cerebellar pathways at the pons' level by cavernous hemangioma. It was assumed that the left cortical hypometabolism was result from damage to thalamo-cortical pathways due to ipsilateral thalamic lesion.
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PMID:[Crossed cerebellar diaschisis and crossed cerebellar atrophy in a patient with a lesion in brain stem]. 204 4

Although cerebral venous malformations have been reported to cause epilepsy, progressive neurological deficits, and hemorrhage, their clinical significance remains controversial. In an attempt to clarify the natural history of the lesion and suggest an appropriate management strategy, the authors review their experience with 30 patients. In four patients with cerebellar venous angioma, an acute episode of ataxia was documented. The coexistence of a cavernous malformation was pathologically confirmed in the two patients who underwent surgery for bleeding presumed caused by the venous angioma. Infarction was shown in two patients and a tumor in two others. Follow-up periods ranged between 18 and 104 months, with only five patients symptomatic at the time of this report. Rebleeding had not occurred, nor had acute episodes of neurological dysfunction been documented. This clinical experience suggests that a venous malformation is frequently associated with other, more symptomatic conditions and is often erroneously identified as the source of the symptoms. Because the nature of the relationship between the venous malformation and the allied conditions remains ambiguous, it is recommended that patients harboring a "symptomatic" venous malformation undergo high-field magnetic resonance imaging to rule out underlying pathology, and that any such pathology be treated independently of the venous malformation.
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PMID:Cerebral venous malformations. 239 88

Computed tomography (CT) allows earlier diagnosis of intracranial cavernous angioma than was formerly possible. However, cerebellar lesions are uncommon. Cerebellar cavernous angioma with hemorrhage was diagnosed in a 54-year-old male who suddenly developed nausea and vomiting, then declined over the following week and developed ataxia and nystagmus. Blood pressure was normal, and he had no history of hypertension or hemorrhagic diathesis. Precontrast CT revealed an irregularly-shaped, hyperdense lesion in the left cerebellum and an associated hematoma. Injection of contrast medium did not significantly enhance the lesion. Vertebral angiography demonstrated only an avascular mass in the cerebellum. A semiliquid hematoma was evacuated and a vascular nodule was removed from the posterior wall of the hematoma cavity. The histopathological diagnosis was cavernous angioma. Nine sufficiently documented reports of cerebellar cavernous angioma are reviewed and the clinical and radiological features of these lesions are discussed.
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PMID:Cavernous angioma of the cerebellum--case report. 247 64

A 23-year-old pregnant woman presented with sudden diplopia, ataxia, hemiparesis, and headache secondary to a brain stem hemorrhage. Magnetic resonance imaging (MRI) revealed a hematoma associated with a probable cavernous hemangioma of the rostral brain stem. In this report, we discuss the MRI findings leading to the preoperative diagnosis, as well as the surgical techniques involved in the successful resection.
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PMID:Surgical excision of a cavernous hemangioma of the rostral brain stem: case report. 320 Mar 80

Two patients with mitochondrial encephalomyopathy (MEP) serve to emphasize the variability of this group of diseases. Cerebral insults, mitochondrial cardiopathy, relapsing ileus, cerebral angioma, ataxia, and myoclonic seizures characterized the first case of an adult man with similar diseases in his family, interpreted as transitional form between mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) and myoclonus epilepsy associated with ragged red fibers (MERRF). The second patient, a floppy infant with cardiomyopathy and myoclonism, statomotoric and mental retardation showed combined defects in mitochondrial respiratory chain at NADH-CoQ reductase and cytochrome c oxidase and a deficiency of carnitine. In both patients neuropathologically criteria of Leigh's syndrome could be demonstrated in the cerebral cortex, in case 2 also clinically. The classificatory problems of the relationships between KSS, MELAS, MERRF, Leigh's as well as Alpers' syndromes are discussed.
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PMID:Mitochondrial myopathies with necrotizing encephalopathy of the Leigh type. 322 73

Basing on the example of two cases, the clinical and morphological variability of mitochondrial encephalomyopathies is demonstrated. Both patients were of short build, and the clinical signs and symptoms were dementia, ataxia, epilepsy and hardness of hearing, whereas signs of myopathy were very mild or absent. Computed tomography showed infratentorial pronounced atrophy of the brain and basal ganglia calcifications, in one case additionally ischemic infarctions, as can be seen in "mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes syndrome" (MELAS). A CT follow-up over 8 years with a progression of the abnormalities parallel to the progressive clinical course is demonstrated. Besides typical "ragged red fibres-myopathy" different abnormalities of mitochondria were seen by the electron microscope. One of the patients died; he had exceptional pathological-anatomical findings with mitochondrial cardiomyopathy, angioma and necrotising encephalopathy of Leigh's type. The two case reports show that in patients with such multisystemic neurological signs and CT-findings mitochondrial encephalomyopathy should be considered and a muscle biopsy should be performed.
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PMID:[Mitochondrial encephalomyopathy: clinical aspects, CT morphology and neuropathology]. 339 29

Spinal cavernous hemangioma is rare, and it is extremely rare for cavernous hemangioma to develop in the cauda equina. There has been only one report of hydrocephalus associated with cavernous hemangioma in the cauda equina. We report a case of cavernous hemangioma in the cauda equina diagnosed on the basis of the headaches due to hydrocephalus. A 67-year-old man was being treated for Parkinson's disease because of tremor of both upper extremities for several years. In December 1991 he complained of occasional headaches. On February 15, 1992 the headaches became severe and frequent, with nausea and vomiting, and his gait became unsteady. Four days later he came to our hospital. Neurological examination revealed fine finger tremor and truncal ataxia. Computerized tomography scanning and magnetic resonance imaging of the head revealed ventricular enlargement, but there were no mass lesions obstructing the cerebrospinal fluid pathway. Lumbar puncture at the L3-L4 level yielded bloody cerebrospinal fluid, and the pressure had increased to 410 mmH2O. Cerebral angiography showed no abnormal findings. Magnetic resonance imaging of the lumbar spine demonstrated an intradural tumor at the level of vertebral body L2. Spinal angiography showed no evidence of abnormal vascularity in the mass at the L2 level. On March 10, 1992, laminectomy at three levels, L1 to L3 was performed, and a well-defined blueberry-like intra-cauda equina tumor 1 cm. in diameter, was removed. One spinal nerve root passed through the tumor. The pathological diagnosis was cavernous hemangioma. After removal of the tumor, the patient's headaches improved, and a follow-up computerized tomography scan six months later showed normal ventricle size.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Cauda equina cavernous hemangioma associated with hydrocephalus--case report]. 754 25

A rare case of extracerebral dural cavernous angioma sited near the sigmoid sinus is reported. This 60 yr old male patient gave history of episodic ataxia of left sided limbs experienced twice on same day and occasional giddiness. Examination did not reveal any findings. A mass was diagnosed on CT Scan following which angiography was carried out. The features matched with those of a meningioma. Retro-sigmoid craniectomy was performed. Occipital artery was coagulated. Tumor was dissected out. Post-operative course of the patient was uneventful. Histopathology revealed that the mass was a cavernous haemangioma.
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PMID:Dural cavernous haemangioma of posterior cranial fossa. 799 2

The authors report 3 cases in which, in addition to venous angioma, other types of vascular malformations coexisted. They discussed the pathological significance of this coexistence, and the treatment to be given in these conditions. Case 1: A 38-year-old man was admitted to the hospital because of progressive headaches of 3 days' duration. X-ray CT revealed a large hematoma in the left cerebellar hemisphere. Vertebral angiogram showed a caput medusae in the venous phase, which is a typical picture of venous angioma. The hematoma was surgically removed and careful inspection of the cavity wall was made. A thick vein and many thin walled dilated venules draining to the vein were observed in the cavity wall. These abnormal vessels were completely removed. Because of reaccumulation of the hematoma and massive edema of the hemisphere, reoperation was performed. On removing a part of the cerebellar hemisphere, a small mass of vascular network was found and removed together with the hematoma. Histologically, the first specimen was a typical venous angioma, and the second one was a arteriovenous malformation. Case 2: A girl 9 years of age was admitted because of headache and left sided ataxia. CT and MRI revealed a multi staged hematoma in the left cerebellar hemisphere. Vertebral angiography, however, failed to demonstrate any kind of vascular malformations. The hematoma was removed with its wall. The histological appearance was compatible with venous angioma. 4 years later she bled again, and reoperation was performed. Histological examination this time revealed a cavernous angioma.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Venous angioma coexisting with other types of cerebrovascular malformations]. 807


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