Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0028738 (nystagmus)
 7,431 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A rare case of the choroid plexus papilloma originating from the extraventricle was reported. A 48-hear-old woman began not to walk well about two years ago. Then, she was complained of vertigo, hearing disturbance of the right ear and nausea. These symptoms gradually increased. On admission she had papilledema, Brun's nystagmus, hearing disturbance of right ear and cerebellar ataxia. Cerebral angiogram and CT scan showed dilated ventricles and a large tumor in the posterior fossa. At operation, tumor was found in extramedullary space at the right cerebello-pontine angle and extended to the foramen magnum. This was removed totally. Histological examination revealed choroid plexus papilloma. Her postoperative course was satisfactory and shunting procedure was not necessary. According to the electron microscopic findings, the ultrastructure of tumor was similar to that of normal choroid plexus. We could not clear the morphological features which were considered essential for overproduction of CSF in the tumor.
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PMID:[A case of choroid plexus papilloma at the right cerebello-pontine angle (author's transl)]. 30 61

Thirty-six patients with benign intracranial hypertension (BIH) were reviewed. Follow-up was obtained on 33 patients (91%) after a mean period of 7 1/2 years. Precipitating factors were found in 27 patients (75%). On admission, 5 patients had retro-ocular pain, especially on eye movements, a complaint not yet described in BIH. Seven patients had nystagmus, two of them horizontal positional nystagmus. It is questionable whether all signs in BIH are caused by the raised CSF pressure. The general outcome was good. Only two patients sustained severe ultimate visual impairment. Both presented with retro-ocular pain and sudden loss of vision on admission. Papilloedema can persist for years in BIH without serious visual impairment. Sometimes "causal" treatment is possible. No symptomatic treatment which is free from complications has been proved to prevent visual failure.
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PMID:Benign intracranial hypertension. A retrospective and follow-up study. 43 Jan

A unique association of Sturge-Weber syndrome and atlanto-occipital assimilation is presented. A 18-year-old male was admitted in emergency because of the sudden severe headache and vomiting. He had vascular nevus in the right half of the face at birth and several episodes of generalized convulsive seizures. On admission craniogram demonstrated calcification in the right occipital area. Neurological examination revealed tenderness in the nuchal region, moderate limitation of cervical mobility in a antero-posterior direction, Bruns-Cushing type nystagmus, bilaterally diminished gag reflex, and positive Romberg's test. Spinal tap showed crystal clear CSF with normal pressure. EEG showed paroxysmal slowing focus in the right parieto-occipital area. Polytomography of the craniovertebral junction demonstrated the unilateral atlanto-occipital assimilation on the left associated with the aplasia of the right posterior arch. Myelography was negative. A right carotid angiography disclosed the dilatation of the basal vein of Rosenthal and abnormal venous vasculature. CT-scan demonstrated the calcified region of the right occipital area more distinctly than the plain roentogenogram, but the enhancement study of the leptomeningeal angioma of the Sturge-Weber syndrome was negative. Never been found this rare association in a review of the literature, the authors discussed the clinical and radiological findings of both diseases.
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PMID:[Sturge-Weber syndrome associated with atlanto-occipital assimilation: a report of a case (author's transl)]. 68 66

A moroccan male aged 26, with Eales's disease since 6 years, develops a low thoracic level paraplegia over 2 months. Examination then also points out an horizontal nystagmus. CSF changes are important: 292 cells/mm3 (96 p. 100 lymphocytes), 3,80 g/l proteins. Slight improvement is obtained by corticosteroid therapy. This case is compared with those of the literature, mostly myelopathies. The pathogenetic problems of immuno-allergic type are discussed.
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PMID:[Eales's disease with neurological involvement (author's transl)]. 74 Nov 33

A rare case of intramedullary schwannoma of the spinal cord has been reported, The patient was a 30-year-old woman, who began to notice weakness in her right leg approximately 6 months prior to admission, followed 4 months later by numbness and weakness of the right arm. The above symptoms were progressively getting worse, and she was admitted to Hokkaido University Hospital on February 23, 1974. Neurological examination revealed slow speech, bilateral horizontal nystagmus, absent gag reflex and weakness of right trapezius muscle. Spasticity was noted in 4 extremities, in addition to right hemiparesis. All deep tendon reflexes were hyperactive, right more than left, with bilateral Hoffmann's and Babinski's signs. Vibration sense was diminished below the level of bilateral iliac crests. A tumor around the foramen magnum was suspected, however plain skull and neck, laminogram of cervical spines, vertebral arteriogram, fractional pneumoencephalogram and myodil myelogram failed to disclose abnormalities. Manometric Queckenstedt test showed a partial block on flexion, with CSF protein of 56 mg/dl. Air myelogram clearly visualized the presence of an intramedullary tumor at the level of the medullo-spinal junction. Subtotal removal of the intramedullary tumor at C1 was performed, which proved to be a schwannoma histologically. 14 such cases are reported in the literature and summarized on Table I, including our case. Clinical features of tumors around the foramen magnum are fairly complexed, and some radiological examinations might not be conclusive. It is stressed that air myelogram is extremely valuable in the diagnosis of lesions around the foramen magnum.
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PMID:[A case of intramedullary spinal schwanoma (author's transl)]. 98 97

A 63-year-old man developed gradually progressive bilateral loss of vision, cerebellar ataxia, and downbeat nystagmus. Visual acuity was 20/400 OD and 20/200 OS, with cecocentral scotomas OU. Fundus examination showed bilateral optic atrophy and a vitreous cellular reaction. MRI of the brain was normal. CSF protein was elevated, with increased IgG levels but no malignant cells. Biopsy of a pulmonary lymph node showed undifferentiated small cell carcinoma. Neoplastic cells were positive for neuron-specific enolase. Serum contained IgG, which reacted with neuronal and glial cytoplasm and processes. IgG reactivity with systemic tissues and the patient's tumor was not different from that observed with control sera. Paraneoplastic optic neuropathy should be considered in patients with unexplained visual loss and malignancy, and our observations suggest a possible immunologic basis for this condition.
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PMID:Optic neuropathy: a rare paraneoplastic syndrome. 132 6

A 70-year-old woman was admitted to our hospital because of rapidly progressive cerebellar ataxia. Neurological examinations showed saccadic eye movement, downbeat nystagmus, scanning speech, proximal dominant muscle weakness and severe truncal and limb ataxia. Based on these clinical features, she was suspected to have paraneoplastic cerebellar degeneration (PCD), although the malignant tumor was not detected through clinical intensive surveys. Her serum and CSF revealed to have anti-Purkinje cell antibodies immunohistochemically, and western blot analysis showed that they reacted with 58 kd band. In view of previous reports of PCD, she was strongly suspected to have gynecological cancers. The trial laparotomy found early stage fallopian tubal cancer, which had not been detected by CT scan, ultrasonogram and MRI. It is important to detect and characterize these autoantibodies found in the PCD patients for early diagnosis and treatment of underlying cancer.
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PMID:[A case of paraneoplastic cerebellar degeneration--success in early detection of cancer by anti-Purkinje cell antibody]. 162 54

An autopsy case of glioblastoma of the cerebellum associated with an intracerebellar hemorrhage and showing CSF seedings is reported. A 26 year-old male was admitted to our hospital with a 10-day history of headache, nausea and vertigo. On admission, disturbance of consciousness (10-20 by JCS), irregular respiration and central fixation of both eyes suggesting increased intracranial pressure and early stage of central herniation were recognized clinically. The cerebellar signs of dysmetria and nystagmus were also observed. CT scan and angiography revealed an avascular large mass in the right cerebellar hemisphere, obstructive hydrocephalus and upward transtentorial herniation. On MRI study, the mass was demonstrated to be a subacute hematoma with a small tumor in its margin. Total removal of the tumor and aspiration of the hematoma were performed. Histological examination revealed a highly cellular and pleomorphic astrocytic tumor with scattered small necrosis and glomeruloid capillary endothelial proliferation, typical of glioblastoma multiforme. During postoperative radiochemotherapy (focal irradiation to the posterior fossa), the tumor showed rapid regrowth and a second look operation was performed. He was readmitted 3 weeks after radiochemotherapy with complaints of severe headache, nausea and lumbago. He then suddenly became dyspnea, tetraplegic and bradycardic. Neuroradiological investigation revealed multiple masses in the suprasellar region, medulla oblongata and the cervical spinal cord, but no recurrence in the cerebellum. Malignant cells were noted on CSF cytology. During chemotherapy for CSF tumor dissemination, his condition deteriorated rapidly and he died 7 months after the onset of symptoms.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Glioblastoma of the cerebellum: report of an autopsy case associated with intratumoral hemorrhage and CSF seedings]. 165 2

A 47-year old female had a fever about 39 degrees C of unknown origin for 2 days. Soon she developed pain in the bilateral lower extremities followed by gait disturbance and vesicorectal disorder. Prednisolone was administered with an improvement. However, she developed paresthesia in the upper extremities 1 month later, and then gradually paraplegia another 5 month later. Nystagmus, painful tonic spasm, facial spasm, and visual disorder also appeared. These symptoms repeatedly exacerbated and remitted with administration of prednisolone. We examined this patient at age 53, CBC, blood chemistry, urinalysis, ECG and chest X-ray were normal. Serum IgG and IgA level were decreased. CSF protein content and IgG level were remarkably increased. EEG showed diffuse theta activities. MRI studies revealed high intensity signals in the putamen, deep frontal and periventricular white matter region. Pulse therapy of methylprednisolone was performed effectively for several times. She died of respiratory and heart failure 6 years after the onset. Autopsy revealed bilateral continuous cystic lesions along the lateral ventricles extending from the frontal tips of anterior horns to the occipital tips of posterior, and further, to the temporal tips of lateral horns; the caudate-callosal angeles (Wetterwinkel) were more severely and widely affected bilaterally. There were also old and fresh demyelinated lesions scattered in the cerebral white matter, brainstem, cerebellum, and spinal cord. Although this case is considered to have typical MS from clinical and pathological findings, there have been only a few reports of MS with such continuous cystic lesions in the cerebral hemispheres as seen in this case.
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PMID:[An autopsy case of multiple sclerosis with bilateral continuous cystic lesions along lateral ventricles and caudate-callosal angles (Wetterwinkel)]. 179 16

A 29-year-old man complained of increasing paraplegia and sphincter disturbances. On admission, he was 23 years old. He had moderate pigmentation of the skin, and his neurological examinations revealed spastic paraplegia, hyperreflexia of both legs with Babinski's signs, a pinprick sensation deficit below the L-1 level, loss of vibration sense in the lower extremities and horizontal nystagmus on lateral gaze. Endocrinological examinations revealed adrenocortical insufficiency. CSF, EEG, EMG, brain-CT and myelography did not show any abnormalities, but metrizamide CT myelography at the low thoracic spinal cord revealed decreased cord diameter. Nerve conduction velocities showed impairment in the tibial and peroneal motor fibers. Auditory brain-stem response revealed elongated III-V interval. A cystometrogram disclosed a reflex neurogenic bladder. In the analysis of the fatty acid component of plasma sphingomyelin, the C26/C22, C25/C22, C24/C22 ratios were found to be increased, and the diagnosis of adrenomyeloneuropathy (AMN) was confirmed. The patient's mother was also found to be asymptomatic carrier of AMN on the basis of long chain fatty acid plasma levels. The MRI performed in his age of 29 years, showed marked spinal atrophy from low cervical to low thoracic regions and mild cerebellar atrophy. This findings seems to correspond with chronic progressive demyelination of spinal white matter such as pyramidal tract and fasciculus gracilis.
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PMID:[A case of adrenomyeloneuropathy with marked spinal cord atrophy on magnetic resonance imaging]. 191 36


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