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PubMed1982

Progressive supranuclear palsy: the relationship between ocular motor dysfunction and psychological test performance.

The performance of patients with progressive supranuclear palsy on visual search and scanning tasks was related to the pattern of ocular deficits observed in these patients during horizontal refixation. Comparisons were made to age-matched normals and patients with Parkinson disease or cerebellar damage. The poor performance of the progressive supranuclear palsy group on visual search and scanning could not be attributed to the restricted range of vertical gaze or the large number of hypometric saccades during horizontal refixation. Instead, we believe that their impaired scanning resulted from the presence of square-wave jerks during attempted fixation.

Bulbar Palsy, Progressive↗
PubMed1985

Celiac disease and spinocerebellar degeneration with normal vitamin E status.

We studied a 47-year-old man with spinocerebellar degeneration and malabsorption due to celiac enteropathy; the serum vitamin E level was normal. The neurologic disorder initially deteriorated despite improvement of small bowel histology on a gluten-free diet and vitamin E therapy, but later stabilized. The etiology of the neurologic disorder in adult celiac disease has not been identified and does not appear to be vitamin E deficiency.

Celiac Disease↗
PubMed1992

Cerebellar diaschisis revisited: pontine hypometabolism and dentate sparing.

A unilateral supratentorial lesion may cause hypometabolism in the contralateral cerebellar hemisphere (crossed cerebellar diaschisis). We analyzed glucose metabolism, measured by PET-FDG, in the posterior fossa in 67 patients (78 PET studies) with primary unilateral supratentorial brain tumors selected for visually obvious metabolic asymmetry in the cerebellar hemispheres. We found that glucose utilization was 17% lower in the contralateral cerebellar cortex (compared with the ipsilateral one), consistent with the selection criterion, and 19% lower in the ipsilateral pons, wherein lie the first order synapses of the corticopontocerebellar pathway. This finding helps to validate the prevalent view that cerebellar diaschisis is due to interruption of afferent input from the corticopontocerebellar pathway. However, glucose metabolism in the contralateral dentate nucleus was relatively preserved--only 2% less than the ipsilateral dentate. This "dentate sparing" suggests preservation of afferent input to the largest of the deep cerebellar nuclei from the Purkinje cells in the cortex, despite interruption of the major excitatory input to the Purkinje cells.

Adolescent↗
PubMed1993

Seronegative, non-neoplastic acute cerebellar degeneration.

Five women are presented with a subacute pure pancerebellar syndrome of ataxia and dysarthria to emphasize that this illness may occur without anti-Purkinje-cell or anti-Yo antibodies, or associated systemic cancer. The syndrome evolved over weeks or less and stabilized in four of the patients. Three had had a preceding infection and one, a rash, but there was no inflammatory response in the spinal fluid. Over the 5-year period during which these patients appeared in an individual general neurology practice, there was only one patient with paraneoplastic cerebellar degeneration. Despite the association of this syndrome with cancer, particularly in women, clinicians will still commonly confront the idiopathic variety.

Adult↗
PubMed1994

The syndrome of 'cerebellar' mutism and subsequent dysarthria.

"Cerebellar" mutism refers to a specific childhood disorder in which a complete but transient loss of speech, followed by dysarthria, occurs after removal of a cerebellar tumor. We present a consecutive series of 15 children with this disorder, which we prefer to designate "mutism and subsequent dysarthria." The conditions in which it develops suggest also an extracerebellar component of cerebellar mutism. Hydrocephalus at presentation, localization of tumor adjacent to the fourth ventricle, and postsurgical edema of the pontine tegmentum are involved in its development.

Astrocytoma↗
PubMed1996

Speech timing in ataxic disorders: sentence production and rapid repetitive articulation.

We studied syllabic timing in patients with ataxia (10 with cerebellar atrophy, 6 with Friedreich's ataxia) under two conditions: in a "natural" sentence production context and in the context of a rapid syllable repetition task. The two tasks included comparable articulatory maneuvers. We measured syllable durations from the speech signal and analyzed variables describing average syllabic rate and within-trial variation of syllable durations. Among the observed measures, slowed syllable repetition was a particularly powerful predictor of the severity of dysarthric impairment. In sentence production, patients often performed at normal syllabic rates. Irregular pacing of syllable repetitions was frequent. Different patterns of between-articulator variation emerged in the two tasks. All patients except one were slower in rapid repetitive articulation than in sentence production. These data suggest that sentence production and rapid repetitive articulation are governed by basically different motor processes. The disproportionate slowing of ataxic patients in the repetitive task can be ascribed to adaptation to novel motor tasks being impaired in cerebellar disease.

Adolescent↗
PubMed1980

Acute cerebellar hemorrhage in childhood: etiology, diagnosis, and treatment.

Four cases of acute cerebellar hemorrhage in childhood are reported. Two were related to the rupture of arteriovenous malformations, one was a hemorrhage within a cerebellar tumor, and one (in a neonate) was of an undetermined etiology. Three of the cases were treated surgically, whereas the neonatal hemorrhage was managed medically. The latter case seems to be the only reported case of acute cerebellar hemorrhage in childhood that resolved spontaneously. Computed tomographpy proved to be invaluable in the diagnosis, management, and follow-up of the patients. Although surgical treatment is indicated when identifiable lesions cause the hemorrhage, nonsurgical approaches may be satisfactory when lesions are not discernible. Our cases, in addition to those reported previously, suggest a better prognosis for children than for adults with acute cerebellar hemorrhage, regardless of the etiology.

Adolescent↗
PubMed1986

Expanding intracerebellar hematoma: a possible clinicopathological entity.

An adult case of intracerebellar hematoma with a definite capsule is presented. The hematoma is thought to have been enlarged by repeated hemorrhages inside the capsule, with consequent progressive neurological symptoms. Intracerebellar hematoma does not always disappear by absorption. On the contrary, it may expand, and symptoms and signs similar to those of cerebellar tumor may subsequently develop. The authors propose that the expanding intracerebellar hematoma is a possible clinicopathological entity with a definite capsule comparable to that of chronic subdural hematoma.

Adult↗
PubMed1986

Surgical management of cerebellar peduncle lesions in children.

Four children with intrinsic cerebellar peduncle lesions (three benign astrocytomas and one histiocytic encephalitis) are reported. Each patient underwent a resection of mass lesions through posterior fossa craniotomy with microsurgical techniques and a surgical laser. None showed permanent neurological deficits postoperatively. A brief anatomical consideration of cerebellar peduncle lesions and the surgical approach used are described.

Astrocytoma↗
PubMed1986

Spontaneous hemorrhage in a mixed glioma of the cerebellum: case report.

An 8-year-old boy presented in coma and was found to have a massive posterior fossa hemorrhage on computed tomographic scan. Autopsy disclosed a large cerebellar hematoma within a mixed glioma containing both juvenile pilocytic astrocytoma and oligodendroglioma. It is postulated that the hemorrhage originated from the oligodendroglial component of the tumor.

Astrocytoma↗
PubMed2005

Treatment of phenylketonuria-associated tremor with deep brain stimulation: case report.

OBJECTIVE AND IMPORTANCE: Phenylketonuria (PKU) is an inborn error of metabolism that causes severe neurological impairment, despite dietary treatment. We present a case of PKU-induced cerebellar tremor treated with deep brain stimulation. There have been no previously reported cases of a patient with a PKU tremor treated with deep brain stimulation. CLINICAL PRESENTATION: A 36-year-old male patient with PKU presented with signs of cerebellar disease including dysmetria, resting tremor, and intention tremor in the left upper extremity. INTERVENTION: A deep brain stimulation electrode was placed in the ventral intermediate nucleus of the right thalamus. CONCLUSION: Immediately after surgery, the patient had nearly complete resolution of intention tremor in the left arm. His resting tremor in the left hand was also greatly improved. The 30-month follow-up examination revealed maintenance of the immediate postoperative improvement.

Adult↗
PubMed2002

Sterile abscess mimicking recurrent tumour in the cerebellopontine angle.

Vestibular schwannomas are the most common tumours encountered in the cerebellopontine angle (CPA) region, accounting for 90 per cent of all lesions. Early pyogenic abscess following surgery and delayed abscesses due to retained foreign bodies have been reported. We describe a case of sterile abscess of unknown aetiology in the CPA region, occurring 13 years after surgical excision of a vestibular schwannoma. The clinical and radiological features were suggestive of recurrent vestibular schwannoma or malignant transformation. We believe this is the first reported case of delayed occurrence of sterile abscess in the CPA region. Further the diagnostic difficulties of such rare lesions occurring in the CPA after vestibular schwannoma surgery are discussed.

Abscess↗
PubMed1991

A case of multiloculated, intracranial epidermoid cyst in a beagle dog.

An intracranial epidermoid cyst was identified within the fourth ventricle of a male 10-month-old beagle dog. The cyst, which exhibited a multilocular structure, was lined by a stratified squamous epithelium and filled with desquamated keratin. In the region of the roof of the fourth ventricle, choroid plexus adherent to the cyst wall and secondary compression of the cerebellum were also seen.

Animals↗
PubMed2005

Pseudotumoural hemicerebellitis: imaging findings in two cases.

Pseudotumoural hemicerebellitis is exceedingly rare. It may closely resemble a tumour either clinically or on imaging, and a high index of suspicion is required to avoid unnecessary surgical procedures. MRI is the most useful diagnostic tool, and reveals a swollen cerebellar hemisphere, hyperintense in T(2) weighted images. A pattern of predominantly pial contrast enhancement, absence of a well-defined mass and regression of the abnormalities in follow up examinations help to rule out malignancy. We report two cases of pseudotumoural cerebellitis (an 11-year-old girl and a 9-year-old boy) studied with CT and MRI.

Cerebellar Diseases↗
PubMed1977

Environmental effects on the central nervous system.

The central nervous system (CNS) is designed to respond to the environment and is peculiarly vulnerable to many of the influences found in the environment. Utilizing an anatomical classification (cortex, cerebellum, peripheral nerves) major toxins and stresses are reviewed with selections from recent references. Selective vulnerability of certain areas to particular toxins is apparent at all levels of the CNS, although the amount of damage produced by any noxious agent depends on the age and genetic substrate of the subject. It is apparent that the effects of certain well known and long respected environmental toxins such as lead, mercury, etc., deserve continued surveillance. In addition, the overwhelming impact on the CNS of social damages such as trauma, alcohol, and tobacco cannot be ignored by environmentalists. The effect of the hospital and therapeutic environment has become apparent in view of increased awareness of iatrogenic disorders. The need for particular laboratory tests, for example, examination of CSF and nerve conduction toxicity studies, is suggested. Epidemics such as the recent solvent neuropathies suggest a need for continued animal studies that are chronic, as well as acute evaluations when predicting the potential toxic effects of industrial compounds.

Auditory Pathways↗