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At least 523 records · Page 29Linked to original sources
PubMed2005

Animal models of generalized dystonia.

Dystonia is a prevalent neurological disorder characterized by abnormal co-contractions of antagonistic muscle groups that produce twisting movements and abnormal postures. The disorder may be inherited, arise sporadically, or result from brain insult. Dystonia is a heterogeneous disorder because patients may exhibit focal or generalized symptoms associated with abnormalities in many brain regions including basal ganglia and cerebellum. Elucidating the pathogenic mechanisms underlying dystonia has therefore been challenging. Animal models of dystonia exhibit similar heterogeneity and are useful for understanding pathogenesis. The neurochemical and neurophysiological abnormalities in rodents with idiopathic generalized dystonia suggest that dysfunctional output from basal ganglia, cerebellum, or from multiple systems is the cause of motor dysfunction. Findings from drug- or toxin-induced dystonia in rodents and nonhuman primates mirror the genetic models. The parallels between dystonia in humans and animals suggest that the models will continue to prove useful in determining pathogenesis. Furthermore, detailed characterization of the existing models of dystonia and the development of new models hold promise for the identification of novel therapeutics.

Animals↗
PubMed1989

Cavernous angioma of the cerebellum--case report.

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.

Cerebellar Diseases↗
PubMed1991

Intracranial cystic hemangiopericytoma--case report.

A rare case of intracranial hemangiopericytoma associated with a large cyst was treated by gross total removal and local irradiation. The tumor has not recurred for 16 months, although the effectiveness of radiation therapy for hemangiopericytoma is unclear. Histological examination of the tumor specimen showed aggregation of the microcystic components, possibly contributing to the cyst formation. Hemangiopericytoma should not be classified as meningioma because of the different neoplastic and cytological properties. Complete surgical removal is essential for this tumor because of its malignancy.

Adult↗
PubMed2001

Metastatic bronchogenic carcinoma with human chorionic gonadotropin production manifesting as cerebellar hemorrhage--case report.

A 58-year-old male presented with a rare case of brain metastatic bronchogenic carcinoma with human chorionic gonadotropin (hCG) production causing cerebellar hemorrhage with symptoms of nausea, vomiting, and headache. Bronchogenic carcinoma manifesting as gynecomastia had been resected a few months previously. Neurological examination revealed left cerebellar ataxia. Neuroimaging showed multiple cerebellar metastases with cerebellar hemorrhage adjacent to the tentorium. Angiography demonstrated tumor staining fed by the hemispheric branch of the left posterior inferior cerebellar artery. Suboccipital craniectomy was performed. The left cerebellar hematoma was evacuated and the tumor was partially removed to prevent massive intraoperative hemorrhage and avoid brain stem injury. Histological examination showed the resected tumor was large cell carcinoma. hCG was detected in the cerebrospinal fluid and was identified by immunohistochemical staining in tumor cells. The primary lesion of bronchogenic carcinoma showed choriocarcinomatous change because the tumor could produce hCG. The choriocarcinomatous cells with higher metastatic potential formed lesions in the brain, and finally intratumoral hemorrhage occurred producing the rapid development of symptoms.

Carcinoma, Bronchogenic↗
PubMed1989

The cerebellum in sagittal plane--anatomic-MR correlation: 2. The cerebellar hemispheres.

Thin (5-mm) sagittal high-field (1.5-T) MR images of the cerebellar hemispheres display (1) the superior, middle, and inferior cerebellar peduncles; (2) the primary white-matter branches to the hemispheric lobules including the central, anterior, and posterior quadrangular, superior and inferior semilunar, gracile, biventer, tonsil, and flocculus; and (3) several finer secondary white-matter branches to individual folia within the lobules. Surface features of the hemispheres including the deeper fissures (e.g., horizontal, posterolateral, inferior posterior, and inferior anterior) and shallower sulci are best delineated on T1-weighted (short TR/short TE) and T2-weighted (long TR/long TE) sequences, which provide greatest contrast between CSF and parenchyma. Correlations of MR studies of three brain specimens and 11 normal volunteers with microtone sections of the anatomic specimens provides criteria for identifying confidently these structures on routine clinical MR. MR should be useful in identifying, localizing, and quantifying cerebellar disease in patients with clinical deficits.

Cerebellum↗
PubMed2002

Malignant squamous degeneration of a cerebellopontine angle epidermoid tumor. Case report.

The authors present the case of a woman with a cerebellopontine angle (CPA) epidermoid cyst that degenerated into a squamous cell carcinoma. Malignant degeneration of an epidermoid cyst is an extremely rare occurrence. Malignant transformation must be considered in the differential diagnosis when new contrast enhancement on imaging studies and progressive neurological deficit are seen in a patient harboring an epidermoid cyst. The patient initially presented with a 10-year history of left trigeminal neuralgia, subacute left-sided hearing loss, and with facial weakness of 3 weeks' duration. Initial magnetic resonance (MR) imaging revealed a left CPA mass, consistent with an epidermoid. There was faint contrast enhancement where the tumor was in contact with the lateral brainstem. A subtotal resection was performed. Histopathological findings were consistent with an epidermoid tumor. One year after initial presentation, the patient's neurological deficit had increased, and follow-up MR imaging demonstrated a large contrast-enhancing tumor filling the left CPA and compressing the brainstem. At repeated surgery a squamous cell carcinoma arising from the previous epidermoid was found. The patient was subsequently treated with external-beam radiotherapy and stereotactic radiosurgery. Her tumor stabilized. Three years and 8 months after the patient's initial presentation, a new area of tumor developed at the torcular Herophili. The patient died shortly thereafter. Malignant squamous degeneration is a rare cause of enhancement on MR images, as is progressive neurological deficit in a patient with an epidermoid. The combination of subtotal resection, external-beam radiotherapy, and stereotactic radiosurgery may be useful for local tumor control but the long-term prognosis is guarded.

Carcinoma, Squamous Cell↗
PubMed1998

Phenytoin: effective but insidious therapy for epilepsy in people with intellectual disability.

Phenytoin (5,5-diphenylhydantoin), which has been in use for 60 years, is still an important antiepileptic drug. Its primary mechanism of action is modulation of the sustained repetitive firing of neurones by direct inhibition and blockage of voltage-gated sodium channels in the neuronal cell membrane, and by delay of cellular reactivation. The plasma protein binding of phenytoin is normally between 90% and 95%. The drug is rapidly distributed from the blood to the tissues and is almost completely metabolized in the liver. The plasma phenytoin concentration normally reaches the steady-state level within 1-2 weeks. The half-life of phenytoin is less than 20 h in low doses, but is prolonged in high doses, newborn infants and elderly people. The half-life is shortened when phenytoin is given concomitantly with an enzyme-inducing drug, such as phenobarbital or carbamazepine. Phenytoin is effective for treating generalized tonic-clonic seizures, partial seizures with or without generalization, and convulsive status epilepticus. Over the years, many new, and even serious, adverse effects of phenytoin have been recognized. Phenytoin encephalopathy, manifesting as cognitive impairment and a cerebellar syndrome, is an important adverse neurological effect, the development of which depends on the saturation kinetics of phenytoin, individual differences in phenytoin metabolism, an inhibitory effect of certain drugs on phenytoin metabolism, or the ability of certain drugs to displace phenytoin from plasma proteins, leading to an increase in the plasma level of unbound phenytoin. Because of its potentially adverse effects, phenytoin is not recommended as the first choice for treating epileptic seizures, except as a co-drug for managing convulsive status epilepticus. In patients with epilepsy who also have intellectual disability, and are susceptible to balance disturbances and cognitive dysfunction, it is wise to replace phenytoin with another drug, such as carbamazepine or oxcarbazepine. The long-term use of phenytoin is not recommended for patients with loss of locomotion, marked cognitive impairment, or symptoms and signs of cerebellar disease. The prevention of phenytoin intoxication, with the subsequent development of phenytoin-induced encephalopathy, depends on careful observation of the patients and frequent monitoring of plasma levels of phenytoin and other concomitantly administered antiepileptic drugs.

Anticonvulsants↗
PubMed1976

[Cerebellar hemangioblastoma with multiple aneurysms].

We have reported a case of cerebellar hemangioblastoma in a 50 year-old Japanese female in which bilateral kidney and ovarian cysts, adrenal adnomatus hyperplasia, and erythrocytosis were observed. Futhermore, an aneurysm not initially observed was detected by cerebral angiography done 23 months after tumor extirpation. We have evaluated the relationship between cerebellar hemangioblastoma and concurrent multiple aneurysms with reference to reported cases.

Cerebellar Diseases↗
PubMedDate not supplied

[Clinical and genetic studies of a family from Peru affected by spinocerebellar ataxia type 7].

INTRODUCTION: Recently the clinical and genetic investigation of cerebellar diseases has experienced an extraordinary advance due to localization of the disorders in different chromosomal loci. This type of investigation has been done in Peru in the last decade, studying a Peruvian family from the Huarochiri province. PATIENTS AND METHODS: A Peruvian family of 65 individuals in five generations, affected by several neurological disturbances associated with ataxia, were studied by clinical, neurological, ophthalmological, neuroradiological and genetic methods. The genetic study was done in the neurogenetics laboratory of the University of Utah (Salt Lake City, United States). RESULTS: The patients showed equilibrium disturbances, gait difficulties, ophthalmoplegia, corticospinal signs, cognitive deficiencies, visual weakness, maculopathy, retinal degeneration, optic nerve atrophy and atrophy of the cerebellum, brain stem and cerebral hemispheres. The DNA in 30 individuals showed, in the neurologically affected, repeated expansion of CAG triplets in the short arm of chromosome 3p. CONCLUSIONS: The findings in this family suggests that they suffer from spinocerebellar ataxia type 7. This could be the first description of the disease in Latin-America.

Adult↗