Cases reported "Cerebellar Diseases"

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1/64. Disorders in cerebellar ocular motor control. II. Macrosaccadic oscillation. An oculographic, control system and clinico-anatomical analysis.

    A distinctive cerebellar ocular motor disorder, macrosaccadic oscillation, evolved simultaneously with an acute cerebellar syndrome in 4 patients, 2 with haemorrhagic metastatic melanoma deep in the vermis, a third with a presumed cerebellar haematoma and a fourth with focal demyelinating disease. Ocular oscillations were conjugate, horizontal, symmetrical, occurred in bursts of several seconds duration, had amplitudes of 30 degrees to 50 degrees, and were evoked whenever the patient attempted to shift visual fixation or pursue a moving target. Photo-electric recordings in one patient with tumour defined features of this disorder of saccadic eye movement: (i) oscillation was composed of saccades, (ii) frequency was 2 Hz, (iii) bursts occurred with amplitude first increasing and then decreasing, (iv) intervals between beginnings of saccades averaged 260 ms and (v) eye position did not exhibit systematic drift during the intersaccadic period. These features documented the inreased gain and instability of the visually guided saccadic system. By using increased feed-forward gain in a sampled-data control model we simulated the pattern of macrosaccadic oscillation. We belive that the acute loss of the calibrator function of the cerebellum accounts for the gain abnormality underlying macrosaccadic oscillation.
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2/64. Acquired convergence-evoked pendular nystagmus in multiple sclerosis.

    Nystagmus seen only with convergence is unusual. We describe four cases of acquired convergence-evoked pendular nystagmus in patients with multiple sclerosis. The nystagmus was horizontal and asymmetric in all patients. Eye movement recordings in one subject showed a conjugate rather than a convergent-divergent relationship of the phase of movement between the two eyes. All patients had evidence of optic neuropathy and cerebellar dysfunction. Occlusion of either eye during fixation of near targets led to divergent drift of the covered eye and a decrease in nystagmus. Intravenous scopolamine reduced nystagmus in one patient. Base-in prisms alleviated symptoms of oscillopsia at near and improving reading visual acuity. Convergence-evoked pendular nystagmus may be more common than currently appreciated, particularly among patients with multiple sclerosis.
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3/64. Infratentorial subdural empyema, pituitary abscess, and septic cavernous sinus thrombophlebitis secondary to paranasal sinusitis: case report.

    OBJECTIVE AND IMPORTANCE: Infratentorial empyema, pituitary abscess, and septic cavernous sinus thrombophlebitis are all rare and potentially lethal conditions. The occurrence of all three in a single patient has not previously been described. We present such a case occurring in a young, otherwise healthy man. CLINICAL PRESENTATION: A 26-year-old man with a remote history of sinusitis developed rapidly progressive headache, fever, right eye pain, swelling, proptosis, and visual impairment. magnetic resonance imaging demonstrated diffuse pansinusitis, including sphenoid sinusitis, and extension of inflammation and infection into the adjacent cavernous sinuses, pituitary gland, and posterior fossa. INTERVENTION: Urgent drainage of the ethmoid and maxillary sinuses was performed; pus was not identified. The patient continued to deteriorate clinically with worsening of visual acuity. Computed tomography of the head performed the next day revealed worsening hydrocephalus and an enlarging posterior fossa subdural empyema. Urgent ventricular drainage and evacuation of the empyema was performed, and subsequently, the patient's clinical course improved. The microbiology results revealed alpha hemolytic streptococcus and coagulase-negative staphylococcus species. The patient survived but during the follow-up period had a blind right eye and pituitary insufficiency. CONCLUSION: Paranasal sinusitis can have devastating intracranial sequelae. Involvement of the adjacent pituitary gland and cavernous sinuses can result in serious neurological morbidity or mortality, and retrograde spread of infection through the basal venous system can result in subdural or parenchymal brain involvement. A high index of suspicion and aggressive medical and surgical treatment are crucial for patient survival, but the morbidity rate remains high. Our patient survived but lost anterior pituitary function and vision in his right eye.
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4/64. 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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keywords = cortex
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5/64. Cerebellar decompensation following a stroke in contralateral posterior parietal cortex.

    We describe here a patient who exhibited cerebellar hypermetria on the left side following a cerebellar ischemia in left cerebellar hemisphere. She subsequently recovered clinically. However, twenty months after cerebellar ischemia, cerebellar symptoms reappeared suddenly. Moreover, kinematic and electromyographic (EMG) abnormalities during fast movements of left wrist were identical to those detected after the initial cerebellar lesion. Surprisingly, the causal lesion of this cerebellar decompensation was found to be a stroke at the level of the right posterior parietal association area.
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keywords = cortex
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6/64. 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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7/64. Neuropsychological consequences of cerebellar tumour resection in children: cerebellar cognitive affective syndrome in a paediatric population.

    Acquired cerebellar lesions in adults have been shown to produce impairments in higher function as exemplified by the cerebellar cognitive affective syndrome. It is not yet known whether similar findings occur in children with acquired cerebellar lesions, and whether developmental factors influence their presentation. In studies to date, survivors of childhood cerebellar tumours who demonstrate long-term deficits in cognitive functions have undergone surgery as well as cranial irradiation or methotrexate treatment. Investigation of the effects of the cerebellar lesion independent of the known deleterious effects of these agents is important for understanding the role of the cerebellum in cognitive and affective development and for informing treatment and rehabilitation strategies. If the cerebellar contribution to cognition and affect is significant, then damage in childhood may influence a wide range of psychological processes, both as an immediate consequence and as these processes fail to develop normally later on. In this study we evaluated neuropsychological data in 19 children who underwent resection of cerebellar tumours but who received neither cranial irradiation nor methotrexate chemotherapy. Impairments were noted in executive function, including planning and sequencing, and in visual-spatial function, expressive language, verbal memory and modulation of affect. These deficits were common and in some cases could be dissociated from motor deficits. Lesions of the vermis in particular were associated with dysregulation of affect. Behavioural deficits were more apparent in older than younger children. These results reveal that clinically relevant neuropsychological changes may occur following cerebellar tumour resection in children. Age at the time of surgery and the site of the cerebellar lesion influence the neurobehavioural outcome. The results of the present study indicate that the cerebellar cognitive affective syndrome is evident in children as well as in adults, and they provide further clinical evidence that the cerebellum is an essential node in the distributed neural circuitry subserving higher-order behaviours.
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8/64. 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 = 19.524133702924
keywords = cortex
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9/64. Cerebellar cortical degeneration disrupts discrimination learning but not delay or trace classical eyeblink conditioning.

    The authors investigated classical eyeblink conditioning in a relatively rare patient, B.R., with extensive cerebellar cortical atrophy and marked sparing of the dentate nucleus. Patient B.R.'s ability to acquire and extinguish simple associations (delay and trace conditioning tasks) as well as her ability to acquire more complex associations (temporal and simple discrimination tasks) were examined. There are 2 primary findings from this study. First, B.R. showed normal acquisition and extinction in delay and trace conditioning. Second, she demonstrated a complete inability to learn associative discriminations, even in the case of a simple 2-tone discrimination within the context of a delay paradigm. The latter finding was unexpected because of the sparing of her deep cerebellar nuclei. These data suggest that the cerebellar cortex, or pathways traversing cerebellar cortex, play an important role in classical eyeblink discrimination learning.
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keywords = cortex
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10/64. Mitochondrial encephalomyopathy with lactic acidosis and stroke like episodes (MELAS) with prominent degeneration of the intestinal wall and cactus-like cerebellar pathology.

    A 67-year-old woman had frequent subacute ileus, hearing difficulty, muscle atrophy and stroke-like episodes. Computed tomography revealed multiple low-density areas, which did not correlate with the vascular supply, in the cerebral cortex. She had metabolic disturbance comprising lactic acidosis and elevated pyruvate level. Her skeletal muscle biopsy specimen showed ragged-red fibers, and mitochondrial dna analysis revealed a point mutation at position 3243, findings consistent with MELAS. Examination of her small intestine revealed a necrotic zone and numerous abnormal large mitochondria in the smooth muscle cells, vascular media and endothelium, and intestinal ganglion cells. The cerebral cortex showed multiple microcystic necrotic foci in cerebral cortex. Cactus-like pathology resembling the changes associated with Menkes' kinky hair disease and torpedoes were observed in the cerebellar purkinje cells. The intestinal dysmotility due to MELAS and cerebellar changes were presumed to be associated with a disturbance of copper metabolism.
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