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)

The authors present two surgically verified cases of cerebellar astrocytoma extending into the fourth ventricular floor. Each patient presented with positional vertigo, and electronystagmography revealed positional nystagmus indicative of a central lesion. The findings in these cases emphasize the need of looking for positional nystagmus in patients with suspected posterior fossa lesions.
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PMID:Positional vertigo with cerebellar astrocytoma. 99 27

The case of a male child with Russel's syndrome due to a pilocytic astrocytoma located in the diencephalic region is presented. The diagnosis was made in the 16th month of age, but symptoms began in the 4th months of life, when he started losing weight. By the time he was admitted weight was 6150g and he was 74cm tall, with an emaciated aspect, no panniculus adiposus, irritated, and with symptoms of intracranial hypertension. There was convergent strabismus, vertical nystagmus of the left eye and bilateral papilledema. Tendinous reflexes were exacerbated and he had spastic tetraparesis. The endocrine evaluation showed a basal raise of GH (23ng/ml), TSH (6.2mUI/1) and prolactin (26ng/ml). The first two hormones did not respond to the acute test with TRH, while prolactin had a poor response. He was submitted to radiotherapy with linear acceleration (total dose of 4000 rads) and surgery, during which the tumor could not be completely removed due to its large size. After 9 months, the child is doing well, with a considerable weight gain (2500g).
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PMID:[Russel's syndrome: diencephalic tumor in a child]. 211 20

A 1-year 11-month-old girl was admitted for losing her weight and gait disturbance. At 4 months of age, she began to become emaciated inspite of normal food intake. Physical and neurological examinations were normal except for a marked lack of subcutaneous fat, irritability and nystagmus. CT scans demonstrated a large tumor occupied in the third ventricle and marked dilatation of the lateral ventricles. Endocrinological studies revealed high levels of plasma growth hormone (GH) in contrast with normal levels of somatomedin-C. The basal value of GH returned to normal with a subnormal response to insulin subsequently after VP-shunt. Then, a pilocytic astrocytoma was partially resected with transcallosal approach. Postoperative course was uneventful and her growth rate returned to normal range. CT scans after radiation therapy of 49 Gy showed marked decrease in size of the tumor. At 3 years and 6 months of age, enlargement of her breast was pointed out although MRI indicated no enlargement of the tumor. Basal value of LH, FSH, E 1 and E 2 elevated and LHRH test showed over-response of LH and FSH. Other hypothalamic-pituitary functions were partially preserved. Case of precocious puberty following diencephalic syndrome associated to the hypothalamic and/or optochiasmatic glioma is quite rare in the previous literature. Mechanisms of diencephalic syndrome and following puberty are unclear. However, endocrinological and radiological findings observed in the present case suggest that hormonal disfunction might be due to the failure of inhibition on GH and LHRH secretion mechanism in the anterior hypothalamus.
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PMID:[Hypothalamic glioma with diencephalic syndrome and following precocious puberty--a case report]. 251 56

A boy with a left-hemispheric cerebellar astrocytoma had upbeat nystagmus exhibiting increasing-velocity slow phases. The nystagmus improved after excision of the tumour.
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PMID:Upbeat nystagmus as an early sign of cerebellar astrocytoma. 279 5

A diencephalic astrocytoma was diagnosed by ultrasonography in a 5 months old girl with nystagmus and emaciation. A 27 months follow-up with ultrasonography, computed tomography and magnetic resonance imaging, showed an initial improvement after irradiation and afterwards the development of complications with ventricular dilatation and parenchymal calcifications.
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PMID:[Diagnosis and follow-up of Russel's diencephalic cachexia by echography, x-ray computed tomography and nuclear magnetic resonance]. 333 Jan 95

Isolated fourth ventricle is an untoward but interesting complication caused by the shunt procedure, which has been recognized since the introduction of CT scanning. Auditory brain stem evoked potentials (BAEPs) were recorded in a patient with isolated fourth ventricle for assessment of brain stem function. Case 1. A 9-year-old boy. He had undergone repair of the lumbosacral meningocele at birth, followed by shunting procedures, including repeated shunt revision, for hydrocephalus which subsequently developed. CT scanning on admission revealed typical findings of isolated fourth ventricle, while neurological examination disclosed no abnormalities. BAEPs on admission revealed normal waves both in amplitude and latency. This patient has been doing well without further shunting procedures. Case 2. A 17-year-old male was admitted with complaints of headache, diplopia, nystagmus and ataxic gait. He had a history of operation for radical removal of cerebellar astrocytoma and lateral ventriculo-peritoneal shunt at 9 years of age. CT scanning on admission revealed a typical isolated fourth ventricle, and fourth ventricle-peritoneal shunt was performed. Postoperative serial BAEPs revealed gradual improvement of brain-stem function. Clinically he has been symptom-free thereafter except for slight nystagmus. Case 3. A 2-year-old female suffering from hydrocephalus developed after neonatal intraventricular hemorrhage and ventriculitis. After repeated revision of a lateral ventriculo-peritoneal shunt, she was admitted in semicoma with vomiting and opisthotonic posture. CT study revealed an isolated fourth ventricle and preoperative BAEPs showed marked abnormalities. Although fourth ventricle-peritoneal shunt was performed, no remarkable change in BAEPs was obtained, and neurological deficit of considerable degree persisted.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Brain stem auditory evoked potentials for assessment of isolated fourth ventricle in three patients]. 387 52

Brain tumors are, after leukemias, the most frequent fatal neoplasms of infancy. The clinical features and symptoms are often markedly different from those observed in the adult forms, according to the peculiar anatomy and behaviour of the child. Persistent headache, vomiting, astenia , behavioral alterations may be the precocious findings. Later, some more specific and suggestive signs such as strabismus, dyplopia , fast head size increase, funduscopic alterations, ataxia, paresis and nystagmus may be observed. On their appearance a prompt diagnostic work-up should be performed. The tumors of the posterior fossa (cerebellar astrocytoma and medulloblastoma, brainstem glyoma , hependimoma in decreasing order of frequency) generally cause precocious symptoms because of the small dimension of the subtentorial space; the presence in this region of several fundamental nuclei and pathways may explain how also small tumors may cause severe deficits. Supratentorial tumors (astrocytoma, malignant glyoma , hependimoma , craniofaringioma ) often show a more prolonged latency and may begin with signs of endocranial hypertension, seizures, or sometimes with hormonal troubles according to the involved anatomic structures. Hypothalamic astrocytoma is responsible of an extreme weight loss as far as to a cachetic status, due to the hyperincretion of GH. Finally, plexus papilloma, dermoid, optic nerve glyoma , oligodendroglyoma , germinoma, teratoma are responsible of a small number of child brain tumors, with different localization and symptomatology.
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PMID:[Clinical course of brain tumors in childhood]. 673 95

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

A case of cerebellar pilocytic astrocytoma is reported. This tumor occurs typically in the first two decades of life and is seldom reported in adults. The 42-year-old patient presented with occipital headaches, nausea, and unsteady gait. Nystagmus and right dysmetria were noted. A CT scan showed a hypodense, nonenhancing, voluminous, right hemispheric cerebellar cyst. Magnetic resonance imaging showed a nodule in the wall of the cyst which became hyperintense with gadolinium. The mass was resected through a small occipital craniotomy. Neuropathological examination revealed a juvenile pilocytic astrocytoma.
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PMID:Pilocytic cerebellar astrocytoma in adults: case report. 849 94

A 6-year-old boy with signs and symptoms of ocular myasthenia gravis had lid nystagmus evoked by horizontal gaze. MR imaging demonstrated an intrinsic midbrain lesion, which was diagnosed by biopsy as a low-grade astrocytoma. In the setting of ocular myasthenia gravis, the finding of lid nystagmus may serve as a useful clinical sign of intrinsic midbrain disease.
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PMID:Lid nystagmus as a sign of intrinsic midbrain disease. 874 61


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