Cases reported "Cerebellar Diseases"

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1/153. Infantile hiv encephalopathy associated with cerebral and cerebellar telangiectases.

    We describe a paediatric case of hiv encephalopathy associated with cerebral and cerebellar telangiectases. Although immunohistochemistry failed to show hiv in the walls of dilated blood vessels, or in their vicinity, brain capillary telangiectases might be an additional complication indirectly related to paediatric hiv infection.
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ranking = 1
keywords = cerebral
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2/153. Rare occurrence of intracerebellar colloid cyst. Case report.

    colloid cysts are rare intracerebral lesions that are preferentially encountered within the third ventricle. There are only a few reports in which colloid cysts are described in other locations such as the fourth ventricle. A symptomatic intracerebellar colloid cyst in a 45-year-old woman is described. The patient presented with headache, gait disturbance, and nausea. Neuroradiological imaging revealed compression of the fourth ventricle, hydrocephalus, and an intracerebellar cystic lesion measuring 4 x 5 cm that had a small peripheral solid portion. The cyst was successfully removed via a paramedian suboccipital approach. Postoperatively, the patient recovered quickly. The findings in the present report represent an additional example of the broad spectrum of cystic lesions encountered in the cerebellum.
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ranking = 0.46365929067222
keywords = cerebral, ventricle
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3/153. Cerebellar and basal ganglion involvement in Langerhans cell histiocytosis.

    Langerhans cell histiocytosis (LCH) is a disease of unknown cause characterised by proliferation of histiocytic granulomas in tissues; the primary cerebral manifestation is diabetes insipidus caused by hypothalamic infiltration. We present a patient in whom, except for the absence of high signal on T1 weighting in the posterior pituitary, consistent with central diabetes insipidus, MRI showed no evidence of hypothalamic involvement by histiocytosis, despite the long duration of the disease. However, there was bilateral, symmetrical involvement of the cerebellum and globus pallidus in addition to a calvarial lesion. High signal in the cerebellar white matter on T2-weighted images may represent demyelination, gliosis and cell loss, as previously reported on pathologic examination.
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ranking = 0.2
keywords = cerebral
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4/153. Diffuse pachygyria with cerebellar hypoplasia: a milder form of microlissencephaly or a new genetic syndrome?

    We report on 2 families with diffuse pachygyria and cerebellar hypoplasia, who presented hypotonia, ataxia, seizures, and developmental delay since infancy. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed decreased gyral formation in the cerebral cortex and marked hypoplasia in the cerebellum. Cerebellar hypoplasia is often associated with type 2 lissencephaly; however, our cases showed no polymicrogyria, and their clinical findings were quite mild compared with those of microlissencephaly. Their characteristic phenotype suggested a new genetic syndrome, which was possibly inherited as an autosomal recessive trait.
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ranking = 0.2
keywords = cerebral
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5/153. Postoperative brainstem high intensity is correlated with poor outcomes for patients with spontaneous cerebellar hemorrhage.

    OBJECTIVE: The outcomes for patients with cerebellar hemorrhage are thought to be influenced by anatomic damage to the brainstem. In this study, we investigated the magnetic resonance imaging findings in the brainstem, to examine the relationship between the degree of brainstem damage and the outcomes for patients with spontaneous cerebellar hemorrhage who are in poor-grade condition. methods: The results for 31 patients with spontaneous cerebellar hemorrhage, with Glasgow coma Scale scores of 8 or less at admission, who underwent magnetic resonance imaging examinations were reviewed. All patients underwent surgical intervention. The patients were divided into two groups according to their glasgow outcome scale scores at the time of discharge, i.e., patients who experienced good recoveries or exhibited moderate disabilities (Group I, n = 8) and patients who exhibited severe disabilities, were in a persistent vegetative state, or had died (Group II, n = 23). We investigated obliteration of the fourth ventricle and the perimesencephalic cistern and the presence of hydrocephalus in initial computed tomographic scans and the presence of areas of high signal intensity in the brainstem in T2-weighted images. RESULTS: Eight patients experienced good outcomes, and 23 patients experienced poor outcomes. The overall mortality rate was 32.3%. There were no significant differences between groups with respect to computed tomographic findings such as hematoma size, but the incidence of high signal intensities in the pons and midbrain in T2-weighted images for Group II was significantly higher than that for Group I (P < 0.01). CONCLUSION: magnetic resonance imaging clearly demonstrated brainstem damage, and high signal intensity in the brainstem was a significant prognostic factor for determining outcomes for patients with spontaneous cerebellar hemorrhage who were in poor-grade condition.
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ranking = 0.087886430224075
keywords = ventricle
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6/153. Cerebellar dysfunction in chronic toluene abuse: beneficial response to amantadine hydrochloride.

    CASE REPORT: A 21-year-old man who had sniffed toluene since the age of 13 presented with a 4-year history of progressive cerebellar dysfunction and visual deterioration. The patient's condition did not improve despite 5 months of abstinence. magnetic resonance imaging revealed cerebral atrophy and hypointensity signals in the white matter and bilaterally in the globus pallidus, thalamus, red nucleus, and substantia nigra. amantadine hydrochloride therapy (100 mg/d, then 200 mg/d) resulted in dramatic improvement of his cerebellar and visual symptoms.
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ranking = 0.2
keywords = cerebral
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7/153. Posterior fossa tension pneumocephalus.

    Posterior fossa tension pneumocephalus (PFTP) is a very rare clinical entity--OFF few case reports available prove how rare. Five patients with PFTP are presented. All were operated on for posterior fossa lesions. One was operated on in the lateral position and the rest in a sitting position. All the patients had postoperative (early) neurological deterioration, and computed tomography (CT) scans revealed PFTP in the left cerebellopontine angle in one case and in the IV ventricle in the rest. air was aspirated from the posterior fossa in one case. All the cases were electively ventilated for 48-72 h. All the patients had an immediate onset of progressive neurological recovery after early intervention and elective ventilation. One patient died 10 days later because of a recurrence of empyema and the development of meningitis. PFTP is a rare but important complication of posterior fossa surgery. Early intervention and elective ventilation can produce a good recovery.
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ranking = 0.087886430224075
keywords = ventricle
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8/153. adult rhombencephalosynapsis. Case report.

    Rhombencephalosynapsis (RS) is a relatively rare developmental disorder of the cerebellum in which the cerebellar hemispheres are fused across the midline without being separated by a cleft or the vermis. The condition may be associated with hydrocephalus and other intracranial and extracranial abnormalities. The authors report on the case of a symptomatic adult who was successfully treated with suboccipital decompression and duraplasty. A 39-year-old woman presented with intractable pain radiating from the thoracolumbar column to the occiput. A general examination yielded normal findings and a neurological examination revealed only subtle ataxia of tandem gait. The patient underwent magnetic resonance (MR) imaging, the results of which revealed an absent cerebellar vermis with fusion of the cerebellum and mild hydrocephalus. A cine-MR image obtained to evaluate her cerebrospinal fluid flow (CSF) revealed attenuated flow in the posterior fossa and cerebral aqueduct. Preoperative intracranial pressure (ICP) monitoring demonstrated no elevation of ICP (mean 4.3 mm Hg). The patient consented to undergo suboccipital craniectomy and duraplasty. Despite an increase in postoperative ICP (mean 10.77 mm Hg; difference from preoperative level according to a t-test, p = 0.002), the patient experienced symptomatic relief, which has persisted for 3 years. One year postoperatively, a cine-MR image was obtained, which revealed improvement in the patient's CSF dynamics. The authors conclude that, although RS may cause altered flow in the adult, their patient has experienced symptomatic relief, suggesting that her pain was related to local pressure in the posterior fossa.
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ranking = 0.2
keywords = cerebral
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9/153. hypoglycemia-induced cerebellar dysfunction and quantitative positron emission tomography study.

    OBJECTIVE: To describe an unusual case of hypoglycemia-induced bilateral cerebellar dysfunction. BACKGROUND: The cerebellum is known to be resistant to hypoglycemia, and selective cerebellar dysfunction caused by hypoglycemia has not been reported. Previous studies showed that the ratio between the rate constants for glucose uptake and phosphorylation (K1 and k3) is reversed in the cerebellum compared with the cerebral cortex; higher K1 in the cerebellum and higher k3 in the cerebral cortex. methods: Quantitative dynamic PET scanning with labeled fluorodeoxyglucose (18F-FDG) was performed to prove altered glucose kinetics in the cerebellum of a patient who presented with episodic cerebellar dysfunction associated with hypoglycemia. Four control subjects underwent the same study. RESULTS: The ratio between K1 and k3 was not reversed in the cerebellum of our patient (K1 = 0.082, k3 = 0.192). On the contrary, the ratio was reversed in the control subjects (mean K1 = 0.109, mean k3 = 0.080). In addition, the patient's cerebellar metabolic rate of glucose (rCMRglu = 27.9 micromol/100 g/minute) and the rate constant of glucose egress (k2 = 0.543) were relatively increased compared with those of control subjects (mean rCMRglu = 21.9 micromol/100 g/minute, mean k2 = 0.352). CONCLUSIONS: In a case of episodic bilateral cerebellar dysfunction caused by hypoglycemia, quantitative dynamic PET study demonstrated decreased glucose uptake-to-utilization ratio and increased leak of glucose in the cerebellum. The cerebellum is not invariably resistant to hypoglycemia.
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ranking = 0.4
keywords = cerebral
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10/153. Therapeutic effectiveness of acetazolamide in hindbrain hernia headache.

    A 43-year-old man had been suffering from exertional headache for 10 years. Sagittal sections on magnetic resonance imaging showed a Chiari type 1 malformation and a cerebellar arachnoid cyst. This syndrome, named hindbrain hernia headache, disappeared with oral acetazolamide. This treatment should be tried in patients with hindbrain hernia headache prior to considering surgical decompression of the foramen magnum.
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ranking = 0.11013186913953
keywords = foramen
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