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)

An adult case of malignant choroid plexus papilloma is very rare. This report is an adult case of malignant choroid plexus papilloma revealed in the lateral ventricle and in the cerebellopontine (CP) angle simultaneously. A 37-year-old man was admitted to the hospital complaining of headache, nausea, and a floating sensation on August 29, 1984. Neurological examination on admission revealed bilateral papilledema, left dysmetria and horizontal nystagmus. CT scan revealed a slightly high density round mass in the right lateral ventricle and a cystic mass with mural nodule in the left CP angle. The intraventricular mass and mural nodule were enhanced moderately and homogeneously. The initial surgery was for removal of the CP angle tumor, and 8 days later removal of the lateral ventricle tumor was carried out. The histology of these tumors was the same and revealed malignant choroid plexus papilloma. Postoperative radiation therapy was carried out 70Gy to the brain (whole brain; 50Gy, focal; 20Gy) and 30Gy to the whole spine. About 2 years later paraparesis, lower cranial nerve palsy, and disturbance of consciousness had progressed gradually. He died of the severe recurrence of the tumor in the brain stem, and multiple dissemination in the spinal cord on September 6, 1987. There was no recurrence of tumor in the right lateral ventricle. This is a very rare case of malignant choroid plexus papilloma which was revealed in both the supra- and infratentorial regions simultaneously. They may have developed independently or they may have arisen through subarachnoid seeding. Radical removal of the tumor is important to prevent recurrence of malignant choroid plexus papilloma.
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PMID:[An adult case of malignant choroid plexus papilloma in the lateral ventricle and the cerebellopontine angle revealed simultaneously]. 221 70

Various types of associated movements of eyeballs with other cranial muscles have been described in the literature. Only a few observations, however, have been reported on the relation of ocular movements and facial muscles innervated by the facial nerve, especially the orbicularis oculi muscles. We report two cases presenting gaze-evoked involuntary contraction of the orbicularis oculi muscle and unilateral eyelid closure. Case 1 was a 38-year-old housewife who was admitted to our hospital because of gait disturbance and sensory deficits below the neck. She had a 5-year history of left facial palsy, disturbed horizontal eye movements, retrobulbar optic neuritis, spastic paraparesis and bladder and rectal disturbances, some of which had relapsed a few times. Neurological abnormalities were summarized as follows: bilateral pale discs, right Horner's sign, horizontal nystagmus, left facial nerve palsy of peripheral type, spastic paraparesis with left-sided predominance, sensory disturbances below the third cervical segment, truncal and limb ataxia, and bladder and rectal disturbances. Gaze-linked synchronized contraction of the left facial muscles was observed and on the left lateral gaze marked spasm of the left orbicularis oculi muscle with occasional lid closure was evoked. Case 2 was a 72-year-old female with cerebellar hemorrhage. Neurological abnormal findings included mild disorientation, meningeal irritation signs, horizontal nystagmus on lateral gaze, reduced response of reflex eye movement to the left, minimal weakness of the left facial muscles, ataxic dysarthria, mild left hemiparesis and hemisensory deficits with face, truncal and limb ataxia. She could move eyeballs to any direction but with effort to the left.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Gaze-evoked eyelid closure. Report of two cases]. 261 99

A nine-day-old female with cerebellar medulloblastoma treated by surgical and radiation therapy is reported. She suffered from vomiting within a few days after birth. Subsequently, her head became enlarged and she was admitted to our hospital. CT scan on admission showed a large mass lesion at the cerebellum expanding to the pineal portion, and marked hydrocephalus. At the age of 14 days, a ventriculo-peritoneal shunt was carried out, and at the age of 67 days, radical resection of the tumor was performed. The histopathological diagnosis was medulloblastoma. Postoperatively, radiation therapy of the brain was carried out. Irradiated dose was 30 Gy on the whole brain and 20 Gy at the location of the lesion. She was discharged at the age of seven months. She remained in good condition with no neurological deficit except for nystagmus. One week later, it was found that she was unable to move her legs and she was readmitted to our hospital. CT scan showed no remarkable change in the intracranial region, but spinal cord swelling was revealed at the Th12-L5 level. Myelography showed a filling defect at the L3-5 level. Radiation therapy of the spinal cord was performed, and the paraparesis gradually improved. However, her general condition was getting worse, and the following CT scan showed intracranial recurrence of the tumor. She deteriorated day by day and at the age of 9.3 months she expired. Brain tumors during the neonatal period are very rare, especially neonatal medulloblastoma. Only 24 cases can be found in the literature.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of neonatal medulloblastoma]. 268 38

A 36 year-old woman beginning with spastic paraparesis at her age of 11 visited us for evaluation of progressive muscular weakness of the distal portions of the upper extremities and difficulty in speaking at her age of 33. The neurological features at the present are as follows; fine horizontal gaze-nystagmus, impaired smooth pursuit ocular movement, highly spastic paraplegia with pes equino-varus necessitating canes and the wheel-chair, highly accentuated PTRs and ATRs associated with positive Babinski's sign, diminished or absent deep reflexes in the upper extremities, moderate muscular wasting with fasciculation on the tongue and distal portions of the upper extremities (rt less than lt). Sensory or cerebellar functions remain normal. No autonomic finding has been manifested. The HTLV-I antibody titers of serum (eg. PA method: x8192 ) and cerebrospinal fluid are highly positive in various methods. That of her mother (no blood-transfusion history) is positive. The provirus genome analysis on peripheral lymphocytes using the Southern blotting method by the cleaving enzyme Psi I was unable to discriminate that of an ATL patient. MRI of the central nervous system revealed higher signal area (short SE) at the C5/6 region and atrophy of C7/8 region. Neither a definite lesion in the lower brain stem, cerebellum nor cerebral hemispheres was identified. The skeletal muscle X-ray CT on the lower extremities revealed the atrophic flexor thighs and the anterior tibial and peroneal muscles. Needle EMG showed the prominent neurogenic changes in the atrophic muscles. Oral prednisolone therapy for four months relieved nystagmus and difficulty in walking, slightly. She, however, discontinued because of its side effect.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[HTLV-I associated encephalo-myelopathy resembling ALS with concurrence of acute promyelocytic leukemia in a member of the relatives]. 274 90

A case of multiple sclerosis (MS) in a 39 year old Japanese female with high antibody titer to human T-lymphotropic virus type I (HTLV-I) in serum and cerebrospinal fluid (CSF) was reported. At the age of 15, gait and urinary disturbances accompanied by sensory impairment in the lower abdomen and lower extremities developed suddenly. The motor disturbance slowly progressed to tetraparesis over a period of 15 years with several remissions. At the age of 28 the patient suffered from an acute loss of visual acuity, unilateral initially, and became blind by the age of 34. On admission to our department in September, 1987, the patient was alert and had spastic paraparesis, the impairment of all sensory modalities below the level of Th 10 and urinary disturbance. Weakness of facial muscles, nystagmus, deviation of the tongue and dysarthria were also noted. By the PA method, the antibody titer to HTLV-I was measured x512 in the serum and x64 in the CSF. Western blotting analysis of the CSF disclosed the bands to p19, p24 and p28 of HTLV-I gag proteins. The CSF contained a few ATL-like atypical cells and showed mild lymphocytosis, but the total protein was not increased. The computed tomography revealed diffuse low density areas in the cerebral white matter. The magnetic resonance imaging disclosed high intensity signal areas in the cerebral white matter and in the dorsolateral portion of the medulla oblongata on the T2 weighted image. The link of HTLV-I infection to the pathogenesis of human demyelinating diseases was discussed.
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PMID:[A case of multiple sclerosis with high CSF antibody titer to HTLV-I]. 275 51

We report two patients with chronic encephalomyelitis due to Borrelia burgdorferi in whom the definite diagnosis was delayed because of atypical clinical features. The first patient presented with chronic spastic paraparesis, slight ataxia and nystagmus of several years' duration. A tentative diagnosis of multiple sclerosis was made in spite of important abnormalities of the CSF biological characteristics. The second patient presented with an acute aphasia and a bilateral Babinski's sign. He was thought to suffer from benign herpetic meningoencephalitis. Several months later, as the patient experienced relapses with cerebellar and spinal cord involvement, falsely positive tests for syphilis were found and an antibiotic treatment was given. High protein content, low glucose levels, pleocytosis and oligoclonal bands were observed in all CSF samples, but the definite diagnosis was based on the detection of serum and CSF antibodies against B. burgdorferi.
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PMID:[Borrelia burgdorferi encephalomyelitis]. 318 97

Since 1924, when ependymomas were first classified as a distinctive glial neoplasm by Bailey, much has been published concerning these tumors, but there are important points of interest that are still not clear. In order to study more fully the clinical and pathologic characteristics of the ependymoma, we identified 62 patients with histologically proven neoplasms. Twenty-two were supratentorial, 21 were infratentorial, and 19 were intramedullary spinal cord tumors. These groups had mean ages of 17, 7, and 41 years, respectively, at the time of first symptoms. The presenting and accompanying symptoms were related to location and included headaches, nausea, visual changes, hemiparesis, and neck, back, and radicular pain. Neurological signs included papilledema, nystagmus, gait disturbance, cranial nerve palsies, altered mental status, paraparesis, and sensory dysfunction. Radiologic modalities of particular importance included computed tomography and myelography. Surgery and radiation therapy were the primary treatment modalities with median survival times from first symptoms being 92, 36, and 117 months for the above groups, respectively. Based on computer-generated survival curves, several characteristics significantly affected survival. These included tumor site, age, and neuraxis metastases. In patients with supratentorial tumors, cranial nerve palsies, microcystic changes, and mitotic figures were important, while in patients with infratentorial tumors, widened sutures, increased head circumference, age, epithelial features, and subependymal features significantly affected survival. Patients who had complete gross resection of a spinal cord tumor had no recurrences or mortality.
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PMID:Ependymomas: a clinicopathologic study. 335 39

A 67-year-old woman began to have difficulty in walking due to spastic paraparesis, sensory impairment in the lower limbs and instability on standing at the age of 59 years. Eight years later, she developed complete spastic paraplegia and urinary incontinence. On examination, she was found to have down-beat nystagmus and ocular hypermetria. Brain CT scan and MRI revealed cerebellar atrophy, predominantly in the vermis. HTLV-I antibody titer was increased in both serum and cerebrospinal fluid (CSF). She was diagnosed as having HAM and placed on prednisolone, salazosulfapyridine and high-dose vitamin C. Her symptoms including gait disturbance, nystagmus and ocular hypermetria improved but did not completely disappear. In parallel with clinical improvement, the antibody titer to HTLV and the ratio of CD4/CD8 lymphocyte subsets in blood decreased. The above observations suggest that the cerebellar signs and cerebellar atrophy share the same etiology with HAM. The presence of nystagmus and prominent cerebellar signs plus the CT and MRI findings strongly suggest that the lesions in HAM are not limited to the spinal cord but extend to the brainstem and cerebellum.
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PMID:[HTLV-I associated myelopathy (HAM) with cerebellar atrophy presenting a down-beat nystagmus]. 766 28

A 32-year-old woman experienced subacute onset of weakness in her left leg, urinary retention and difficulty in extending her right middle and third finger. She subsequently suffered episodes of myelopathy, optic neuritis and cerebellar ataxia over a period of several years. Brain MRI showed multiple areas of high signal intensity on T2-weighted images, consistent with multiple sclerosis (MS). However spinal MRI revealed no abnormal findings. In her most recent episode, at age 40 she developed paraparesis. Neurologic examination revealed down beat nystagmus on gazing to the right, horizontal jerk nystagmus gazing to the left, weakness of the right middle and third fingers and paraparesis associated with spasticity of the right leg. Sensory disturbance below C3 and diminished vibration and position sense in both legs were also observed. The patient could not stand or walk, and urinary disturbance was present. Spinal MRI revealed syrinx formation at the level of vertebral bodies C2 to C6. The syrinx within the cervical cord diminished in size after four months, but the patient was unable to walk unaided and had moderate sensory disturbance as before. This finding suggests that the prognosis of MS with syrinx formation following repeated episodes of myelopathy is not always favorable. We believe that functional recovery in MS with syringomyelia is affected by the severity of the demyelination and/or gliosis caused by MS rather than by the presence of the syrinx.
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PMID:[Multiple sclerosis with syringomyelia--case report]. 813 3

We present a female patient with typical third stage neuroborreliosis with progressive chronic encephalomyelitis. One month after a tick bite, in the first stage of Lyme disease, she had myalgias during ten days and after one year polyarthralgias and polyarthritis. Neurological problems occurred 15 years after the tick bite with headache, nystagmus, intentional tremor and spastic paraparesis with sphincter disturbances. Etiological diagnosis was established after three years. Cytobiochemical findings in cerebrospinal liquor were normal but oligoclonal IgG bands were found. Fluorescent antibody test was positive in serum (1:75) as well as ELISA (1:447). The patient reacted favourably to intravenous crystal penicillin 20 x 10(6) units daily during 18 days. Till now, she is in remission and has only mild paresis of the left leg.
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PMID:[Chronic encephalomyelitis caused by Borrelia burgdorferi. Case report]. 826 11


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