Cases reported "Cerebellar Diseases"

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1/48. Vascular changes in tuberculous meningoencephalitis.

    Our report refers two cases of tuberculous encephalomeningitis which differ in the course and pathological changes. In case 1 blood vessels showed features of peri, endo-, or panvasculites. In some vessels endothelium proliferation leading to the stenosis or obliteration of the vascular lumen was observed. necrosis was an effect of vessels occlusion. In case 2 many fewer vessel were involved in onflammation process. Vascular changes were also less extensive and were observed more rarely. Tuberculous infection often caused less tissue lesions than vascular changes. Different pathological changes probably depend on the type and virulence of Myobacterium tuberculosis and on the host immune response to the infection.
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2/48. 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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3/48. Postpartum cerebellar herniation in von Hippel-Lindau syndrome.

    PURPOSE: To describe exacerbation during pregnancy of cerebellar hemangioblastoma in von Hippel-Lindau syndrome. METHOD: Case-report. A 21-year-old woman with von Hippel-Lindau syndrome was found on routine ocular examination to have severe papilledema 1 week after giving birth. RESULTS: Immediate magnetic resonance imaging disclosed a large cerebellar cyst from hemangioblastoma causing cerebellar tonsillar herniation. Immediate neurosurgical intervention was life saving. CONCLUSION: Worsening of intracranial hemangioblastoma during pregnancy in cases of von Hippel-Lindau syndrome should be realized and periodic neurologic and ophthalmologic observation is warranted.
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4/48. Swallowing difficulties for cerebellar stroke may recover beyond three years.

    Swallowing disorders after stroke or skull base surgery can be life threatening. Although late recovery can occur, it remains poorly documented. We report a case of a 54-year-old woman with dysphagia resulting from a cerebellar stroke with hemorrhage that was evacuated through craniotomy. Swallowing difficulties were assessed by a videoendoscopic swallowing study. She presented with disruption of swallow initiation and impairment of the pharyngeal stage, resulting in hypopharyngeal stasis and penetration with aspiration. Supportive swallowing therapy was conducted with careful reeducation to assist initiation of the pharyngeal stage as well as development of compensatory postural technique. Initial improvement was very slow but became rapidly progressive from the 31st month after the stroke. By the 34th month, oral feeding was possible without aspiration. This case demonstrates that improvement in swallowing function can be expected even 3 years after stroke or skull base surgery. Determination of predictive factors for late functional recovery is of great importance and should be the focus of further investigation.
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5/48. Isolated cerebellar involvement in Rosai-Dorfman disease: case report.

    OBJECTIVE AND IMPORTANCE: Sinus histiocytosis or Rosai-Dorfman disease (RDD) is a rare but well-recognized disorder characterized by an unusual proliferation of histiocytic cells. Intracranial localization is a rare manifestation of RDD. Only three cases of localization in the posterior fossa have been reported in the literature. The present report describes the first case, to our knowledge, of cerebellar localization of RDD. CLINICAL PRESENTATION: A 67-year-old woman was admitted to our institution with a 5-month history of cerebellar ataxia. Her medical history was unremarkable. The patient was alert and cooperative. No cranial nerve deficits were evident; Romberg positivity to the left side was recorded. No cutaneous abnormalities, lymphadenopathy, or hepatosplenomegaly were revealed by physical examination. Routine hematological and biochemical studies were normal except for the erythrocyte sedimentation rate, which was elevated. Radiologically, the lesion appeared as a well-defined and avascular mass in the right cerebellar lobe. meningioma was considered the most likely diagnosis. TECHNIQUE: The patient underwent a suboccipital craniotomy with complete excision of the lesion. Microscopic examination of the operative specimen revealed the presence of a mixed cellular population with predominant mature histiocytes. A peculiar feature was the presence of lymphocytes and monocytes within the cytoplasm of histiocytes (emperipolesis). Immunohistochemical study of the histiocytes revealed strong positivity for S-100, CD-68 antigen, and vimentin. CONCLUSION: Involvement of the central nervous system in RDD appears to have a benign prognosis, especially in the absence of nodal diseases. Surgery is essential for diagnosis, and, when total removal is achieved, the outcome is generally good without risk of recurrence.
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6/48. hemifacial spasm or subcortical epilepsy?

    A child with Goldenhar's syndrome presented at about one week of age with stereotyped, repetitive paroxysmal episodes of hemifacial contraction, suggestive of partial seizures or hemifacial spasm. Later in life he also developed independent, permanent abnormal eye and chin movements identical in rhythm to those seen in myorhythmias, suggesting involvement of the dentato-olivary pathway. MRI demonstrated a hamartomatous lesion at the level of the pontomedullary junction. We speculate that the nature of the lesion could be responsible for the partial seizures mimicking hemifacial spasm and that because of its location, this same lesion could also be implicated in the genesis of myorhythmias. The presence of a hamartomatous lesion in a region affected by the abnormal development of the first and second branchial arches is not fortuitous.
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7/48. Sudden death from fulminant acute cerebellitis.

    Acute cerebellitis is postulated to result from viral and/or autoimmune etiologies. This disease has been reported to have a variable course. We report a case of sudden death from acute fulminant cerebellitis in a 13-year-old ballet dancer. Serial CT and MRI demonstrated rapid progression of the disease. Histopathologic correlation is provided. The etiologies, clinical course, therapeutic interventions and postmortem evaluation of this potentially life-threatening condition are briefly reviewed.
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8/48. Successful therapy combined with surgery for severe post-transplant nocardiosis.

    We report a case of a 35-year-old man with nocardiosis infection involving soft tissue and the central nervous system who had received a cadaveric donor kidney. The patient was admitted with fever, malaise and right shoulder pain. Soft tissue abscess was seen on ultrasound examination. It was presumed due to gram ( ) microorganisms, so 4 g day (IV) ampicillin/sulbactam was started empirically once the abscess was drained. nocardia asteroides was found in the pus specimen. On the second day in hospital, severe headache, ataxia and signs of meningeal irritation appeared. The cranial CT showed two intracranial abscesses in the frontal lobe and cerebellum. We assumed nocardia asteroides was the infective agent for the cerebral abscesses, so antibiotic therapy was switched to trimethoprim-sulphamethox-asole (3x160/800 mg/d). nausea and vomiting occurred on the fifth day of therapy, improving after drainage from the frontal abscess. However, these complaints recurred five days later. CT showed cerebellar abscess had become bigger. The patient's complaints improved after the second surgical drainage. N. asteroides was again grown in the aspiration fluids of both cerebral abscesses. Complete regression of the abscesses was seen in the CT after two months. Co-trimoxazole was continued for six months then withdrawn. Graft dysfunction was not observed. Early medical and surgical interventions may be life-saving in this potentially lethal disease.
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9/48. Crossed cerebral - cerebellar diaschisis: MRI evaluation.

    MRI, done later in life, in two patients with infantile hemiplegia syndrome showed significant volume loss in the cerebellar hemisphere contralateral to the side of the affected cerebrum. The cerebellar volume loss seemed to correlate with the degree of volume loss in the contralateral cerebral hemisphere. These observations provide morphological evidence of the phenomenon of crossed cerebral-cerebellar diaschisis (CCD). functional neuroimaging studies in support of the concept of CCD has been critically reviewed.
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10/48. Posterior fossa decompression for life-threatening tonsillar herniation in patients with gliomatosis cerebri: report of three cases.

    OBJECTIVE AND IMPORTANCE: Gliomatosis cerebri (GC) is a rare type of primary brain tumor that diffusely infiltrates more than two lobes of the brain while the normal cerebral architecture is maintained. To the best of our knowledge, the association between an acquired tonsillar herniation and GC has never been reported. In this article, we describe three patients with progressive gliomatosis of the cerebellar hemispheres who subsequently showed signs and symptoms secondary to tonsillar herniation. Early recognition of this potentially life-threatening complication allowed us to recommend prompt surgical intervention. CLINICAL PRESENTATION: One patient with primary, or Type I, GC presented with suboccipital headaches, and two patients with secondary, or Type II, GC presented with the signs and symptoms of progressive myelopathy. Serial imaging studies demonstrated progressive involvement of the cerebellum, descent of the cerebellar tonsils through the foramen magnum, and cervicomedullary spinal cord compression. INTERVENTION: Once the tonsillar herniation was recognized, all three patients underwent posterior fossa decompression, a cervical laminectomy to the lowest level of the tonsillar herniation, and duraplasty. All three patients experienced immediate improvement in their conditions. CONCLUSION: Early recognition of tonsillar herniation, a possibly overlooked cause of death in patients with GC, allows for early surgical intervention as a potentially lifesaving procedure and significant improvement in the patient's condition.
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