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)

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

A 50-year-old woman had primary position upbeating nystagmus, abnormalities of the lower cranial nerves, mild cerebellar signs, and CSF lymphocytosis. CT and MRI showed enlargement of the pons. Eye movement recordings revealed impaired pursuit and optokinetic nystagmus, and visual-vestibular interactions were absent. The patient improved spontaneously.
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PMID:Atypical brainstem encephalitis: magnetic resonance imaging and oculographic features. 387 23

A case, a 18-year-old male, of an endodermal sinus tumor (yolk sac tumor) in the fourth ventricle, was reported. The patient had a month history of headache, vomiting and gait disturbance prior to the hospitalization, when he admitted to our service he was in lethargic condition with left cerebellar ataxia and horizontal nystagmus. Lumbar tap revealed clear CSF under normal pressure of 110 mm H2O with the CSF protein of 432.5 mg/dl and cell count of 147/3. The vertebral angiography demonstrated space occupying lesion in the posterior fossa. Plain CT demonstrated only disappearance of the fourth ventricle and slightly dilated bilateral ventricles and third ventricle. However diffuse high density area around the fourth ventricle was demonstrated and the wall of bilateral anterior horn was slightly enhanced, after injection of contrast media. There was no other abnormal findings around the pineal region. Suboccipital craniectomy was performed and the tumor was totally removed macroscopically. The tumor was situated in th floor of the fourth ventricle and infiltrated into the fourth ventricular wall and th adjacent cerebellar tissue. The tumor was with soft, greyish color and extremely vascular. Histologically the tumor was diagnosed as endodermal sinus tumor according to Teilum's classification. There were stellate cells arranged in a loose with vacuolated network which formed cystic cavities and a complicated network of honeycomb appearance with a system of communicating cavities and channels. Various size of intra- and extracellular PAS-positive hyaline globules were also seen. Glomerular-like structure (Schiller-Duval body) was not observed. Immunoperoxidase study clearly demonstrated the presence of intra- and extracytoplasmic alpha-fetoprotein granules in the tumor tissue. The amount of the serum alpha-fetoprotein, measured by radioimmunoassay, showed 400 ng/ml. After irradiation in the posterior fossa (5000 rad) the patient was discharged. Three months later, follow up CT demonstrated small high density area in the anterior horn of the left lateral ventricle, so he was rehospitalised. Irradiation in the whole brain was again administered. The tumor was very radiosensitive. CT, after 800 rad, demonstrated complete disappearance of the tumor. After irradiation totally (3000 rad), he discharged with left cerebellar ataxia.
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PMID:[Primary endodermal sinus tumor of the fourth ventricle (author's transl)]. 616 17

A 17 year old high school boy experienced fever and diarrhea, which subsided within 4 days by appropriate medications. Six days later, however, he developed unsteadiness and limb spasm. On the morning of admission, he was found to have drowsiness, dysarthria, gait disturbance and involuntary jerks. When he was brought to the hospital, he was lethargic but could follow simple verbal commands. Frequent involuntary movements manifested by facial grimacings, limb spasms and twitchings with dystonic features were seen. Decorticate posturing was readily elicited by painful stimuli. There was no meningeal irritation sign or gross sensory impairment. The deep tendon reflexes were symmetrically exaggerated with bilateral Babinski signs. Bilateral lateral rectus muscle weakness was found together with mild ptosis and upward gaze limitation. Nystagmus was not present and the funduscopic examination was normal. Immediately he was placed on anticonvulsants, steroid hormone, gamma-globulin and antibiotics as well. A brain CT scan and a CSF examination revealed no abnormality. Meanwhile he continued to show a progressive deterioration associated with fever and status epilepticus, and within 24 hours he lapsed into coma in decorticate posture. An EEG obtained at the 3rd hospital day was compatible with spindle coma. In spite of aggressive treatment he remained febrile and comatous. Therefore, vidarabine (adenine arabinoside) was initiated from the 3rd hospital day for 5 days. Then he began to groan and show frequent choreic movements. For the subsequent 2 weeks he made a slow recovery.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of brain stem encephalitis with complete recovery (Bickerstaff's encephalitis)]. 620 73

The case of a 28-year-old patient with refractory Klebsiella meningitis after surgical removal of an angioblastoma in the left cerebellar hemisphere (Lindau's tumour) as described in this article, shows the significance of immunoglobulin therapy in view of the synergistic efficacy increase of antibiotics and immunoglobulin. There were 4 meningitis relapses in a period of 8 months, each time 4 days after discontinuation of antibiotics, the therapy consisting of antibiotics only (penicillin G, gentamycin, lamoxactam, cefotaxim and chloramphenicol). These antibiotics, which were applied in accordance with the antibiogram, were discontinued each 16-32 days after the patient had become afebrile and CSF was free from bacteria. In spite of this, further meningitis relapses followed even when the CSF cell count dropped from 12000/3 to 32/3 cells. An attempt to apply gentamycin suboccipitally failed, since diplopia, nystagmus and headache occurred after a single application. The combination of chloramphenicol orally and immunoglobulin intravenously resulted for the first time in complete freedom from bacteria in the CSF. A single intrathecal application of 1 g immunoglobulin caused a temporary increase in CSF cells to 1200/3 and an anal temperature increase from 37% to 38% C. Urticaria exanthema was observed twice as a side effect of immunoglobulin therapy.
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PMID:[Effect of immunoglobulins on the course of therapy-resistant Klebsiella meningitis]. 654 61

A term of 'disproportionately large, communicating fourth ventricle' (DLCFV) was first proposed by in Harwood-Nash in 1980. It is somewhat different from the well known clinical entity of 'isolated or trapped fourth ventricle', because of apparent patency of aqueductal canal. Two cases of typical DLCFV encountered in our clinic were described. First patient was a 24 year old man in whom this condition developed following operations for lumber disc and second patient was 22 year old woman in whom the disease developed after subarachnoid hemorrhage. In both cases, main symptoms were attributable to hydrocephalus but three posterior fossa symptoms, nystagmus, Parinaud' sign and truncal ataxia were also characteristic. On the CT scan, the fourth ventricle was extraordinarily enlarged. Patency of the aqueductal canal was demonstrated by air study or Conray and Metrizamide ventriculography. On the other hand, occlusion was demonstrated or highly suspected in or near the foramina Magendie and Luschka. After a routine ventriculo-peritoneal shunt operation, the fourth ventricle decreased in size and the symptoms were immediately relieved. Plausible explanation for mechanism involved in occurrence of DLCFV were (1) occlusion process in or near the fourth ventricle outlets seems to be crucial in this pathologic condition. Collision of CSF pulse waves against the obstruction may yield a water hammer effect on the fourth ventricle. (2) abnormal weakness of the brain stem parenchyma around the fourth ventricle to CSF pressure may be another contributory factor.
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PMID:[Disproportionately large communicating fourth ventricle--report of 2 cases]. 660 22

Primary amebic meningoencephalitis and granulomatous amebic encephalitis are well recognized clinicopathological entities caused by free-living amebas. Associated arteritis and "mycotic aneurysms" with infiltration of intracranial arteries by lymphocytes, amebic trophozoites and cysts have not been previously reported. A 26-month-old girl had a 3-week history of encephalitis, characterized, initially, by vomiting and low-grade fever. Subsequently, she developed ataxia, generalized weakness, lethargy, and esotropia. The first CSF showed 490 RBC/microliters, 705 WBC/microliters with 90% mononuclears. Her pupils reacted briskly to light. Moderate nuchal rigidity, nystagmus, fixed downward gaze, anisocoria, bilateral 6th nerve palsy, left arm monoparesis and left Babinski were present. CAT scan revealed slight symmetrical dilatation of anterior horns of lateral ventricles and an area of abnormal enhancement above the 3rd ventricle. She died 14 days after admission, 5 weeks after onset of symptoms. The brain showed focal necrotizing encephalopathy, involving thalami, cerebellum, brain stem, and cervical and upper thoracic spinal cord. Numerous free-living amebic trophozoites and cysts were present within a chronic granulomatous encephalitis. There were trombosis of basilar, posterior cerebral, and vertebral arteries with profuse chronic panarteritis, fibrinoid necrosis, and mycotic aneurysms.
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PMID:Granulomatous encephalitis, intracranial arteritis, and mycotic aneurysm due to a free-living ameba. 689 86

Fifty-six patients with cysticercosis and neuro-ophthalmic signs were examined by me during an 11-year period. Their symptoms generally reflected increased intracranial pressure caused by hydrocephalus, subacute meningitis, cerebral masses or a combination of these effects. Papilledema (37 patients) was the commonest sign, with pupillary abnormalities (16 patients) and nystagmus (12 patients) frequently encountered. Cysticercosis is a relatively benign, although episodic, disease, as long as secondary obstructive hydrocephalus is promptly diagnosed and treated by CSF shunting procedures.
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PMID:Neuro-ophthalmologic signs and symptoms of cysticercosis. 711 71

A 28-year old male was admitted to Musashino Red Cross Hospital on June 21, 1975, because of symptoms of increased intracranial pressure and cerebellar dysfunction. Thirteen months prior to admission he had a mild fever, tremor of right arm, headache, nausea and unsteady gait, but made a gradual recovery in about 40 days. A month prior to admission he had unsteady gate again wit dizziness, photophobia and lacrimation. Gait disturbance aggravated and he was admitted to another hospital, where he developed recent memory disturbance and cloudiness of consciousness. Spinal tap revealed initial pressure of 280 mm CSF. So a mass lesion possibly in the posterior fossa was suspected and the patient was referred to the neurosurgical department of musashino Red Cross Hospital. On admission he was moderately disorientated and disturbed in recent memory. Wide based gait, horizontal and vertical nystagmus were also noted. Angiography revealed rounding of the curve of the pericallosal artery but no space occupying lesions. External ventricular drainage was performed on July 25, 1975. After the operation, his orientation improved without change in dizziness, nystagmus and recent memory disturbance. Ventriculography showed hydrocephalus with cisternography revealed a block at the basal cisterns. PPDs was negative and typical sarcoid tubercles were found in the biopsy specimen of the cervical lymphnode. Kveim test was positive. But repeated chest roentgenogram failed to show bilateral hilar lymphadenopathy, or other changes consistent with pulmonary sarcoidosis. Steroid therapy resulted in marked symptomatic improvement.
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PMID:[A case of CNS sarcoidosis -case report of hydrocephalus due to mechanical obstruction secondary to sarcoid granulomata at the outlet of the fourth ventricle (author's transl)]. 723 30

Two cases of the diencephalic syndrome were reported with hormonal studies. The first case was initially admitted at her age of 4 years and 10 months old. Progressive visual disturbance and horizontal nystagmus had developed one year prior to the admission. Her weight gain had stopped since 10 months old. Partial removal of the hypothalamic pilocytic astrocytoma and 5,000 rad of lineac radiotherapy brought satisfactory effects on her previous symptoms. She was readmitted, however, at her age of 8 years and 10 monts old because of precocious puberty. Her breast development was in Tanner's grade 2, and her bone maturation was equivalent to one of 11 years old. Hormonal studies showed decreased urine 17-OHCS and 17-KS, elevated serum LH, E1 and E2. The CT scan disclosed some enhanced area in the vicinity of the optic chiasm, which had not changed for the last one year. The second case was admitted at his age of 10 years and 9 months old because of progressive marked emaciation of 6 months' duration. Parinaud's sign was only positive neurological finding. The CT verified enhanced areas in the pineal and chiasm. Cytological study of CSF gave the diagnosis of germinoma. Hormonal studies showed decreased urine 17-OHCS and 17-KS, decreased T3, T4, ACTH, and increased PRL as well as increased HGH. Local lineac irradiation by 5,500 rads was given, during which the enhanced area in CT disappeared and his weight gain started. Increased HGH became normalized but paradoxical response of HGH to GTT remained same.
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PMID:[Two cases of the diencephalic syndrome were reported with hormonal studies (author's transl)]. 724 42


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