Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 7-month-old infant had the infantile spasm syndrome, consisting of flexor and extensor spasms, developmental delay, and hypsarrhythmia. Corticotropin produced delay, and hypsarrhythmia. Corticotropin produced improvement in the clinical symptoms and reverted the generalized electroencephalographic abnormalities to more focal ones. Removal of a choroid plexus papilloma of the left lateral ventricle was followed by clinical recovery. One year later the child was normal developmentally and neurologically and was seizure free on anticonvulsant therapy.
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PMID:Choroid plexus papilloma and infantile spasms. 44 63

We described a 5-month-old girl with Aicardi syndrome accompanied by auditory disturbance and multiple brain tumors. She was admitted to our hospital because she suffered from intractable flexor spasms. Physical examination revealed craniofacial asymmetry, left auricular deformity, scoliosis, and remarkable hypotonia with psychomotor retardation. Abnormal ophthalmological findings included chorioretinopathy with pale and round-shaped peripapillary lacunae, and there was modified hypsarrhythmia in her EEG. MRI revealed multiple brain tumors in the 3rd and the lateral ventricles which are considered to be choroid plexus papilloma with agenesis of the corpus callosum. ACTH therapy was administered because of the intractable seizures. After ACTH therapy, the thresholds of waves I and V were much improved. The interpeak latency of waves I-V of the left ear and the peak latency of wave I of the right ear had been lengthened. Acoustic reflex with contralateral stimulation showed no response in the left ear. These findings indicate that the auditory system is also involved in the Aicardi syndrome and that ACTH is effective for its dysfunction.
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PMID:Aicardi syndrome accompanied by auditory disturbance and multiple brain tumors. 166 49

A pigmented choroid plexus papilloma was found in the left lateral ventricle of a 15-year-old white boy with a 10-year history of seizures. Grossly the lesion appeared finely lobular, orangish brown, rubbery, gritty, and friable. Microscopic examination revealed delicate papillary formations reminiscent of normal choroid plexus, with simple cuboidal to columnar epithelium overlying fibrovascular cores. Light- and electron-microscopic studies demonstrated that the neoplastic cell pigment consisted of both neuromelanin and lipofuscin. To our knowledge, this is the first reported case of neuromelanin pigmentation within a choroid plexus papilloma.
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PMID:Pigmented choroid plexus papilloma. 219 7

The authors report the successful total removal of a rare intracranial tumor in the right lateral ventricle of a 15-year-old girl with epileptic seizures. Histopathological examination showed a benign tumor formed by mature cartilage and choroid plexus papilloma. From our review of the literature, a mixed tumor with these histological features has not been reported previously in this location. The diagnosis, surgical approach, etiology, and prognosis of this lesion are discussed.
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PMID:Choroid plexus papilloma with chondroma: case report. 373 8

The incidence of choroid plexus papilloma is about 0.5% of all intracranial tumors, but choroid plexus papilloma in the third ventricle is rare. Fortuna reported 56 cases of choroid plexus papilloma of the third ventricle in 1979. Among them, the neoplasm of the third ventricle in the neonatal period was not found. This report is a case of choroid plexus papilloma of the third ventricle in the neonatal period. A 34 day-old female was admitted to our service with complaints of head enlargement, vomiting, and convulsive seizures on January 8, 1981. Enlarged head had been noticed by her family within the first two weeks. On admission, a marked congenital hydrocephalus was diagnosed by CT scan with symmetrical dilated ventricles and no abnormal high or low density area, and V-P shunt was performed on the next day. But several days later, she suffered from progressive abdominal distension, which was disclosed due to CSF overproduction by a choroid plexus papilloma. When the tumor was recognised by enhanced CT scan, her general condition was too poor to attempt a surgical procedure, and she died on March, 19, 1981. Histologically the neoplasm was a typical choroid plexus papilloma of the third ventricle. So far as we know, this case is the first one of the choroid plexus papilloma of the third ventricle in the neonatal period.
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PMID:[A choroid plexus papilloma of the third ventricle in the neonatal period--a case report]. 662 95

The reversal of diastolic cerebral blood flow has been regarded as a characteristic waveform of brain death and a useful confirming sign. We report 2 patients who had diastolic flow reversal but survived. One, a 1-month-old boy with status epilepticus, had reversal of diastolic cerebral blood flow detected by Doppler ultrasound soon after admission. Reversal disappeared after medical management for increased intracranial pressure and seizure control. He recovered without sequelae. The other, a 6-month-old girl with choroid plexus papilloma, had reversal of diastolic flow during abrupt clinical deterioration. Emergent surgical removal of the tumor was performed and she survived with hemiparesis and psychomotor retardation. Our patients demonstrated that even in the presence of diastolic reversal of cerebral blood flow, prompt and effective treatment can avoid a fatal outcome.
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PMID:Reversal of diastolic cerebral blood flow in infants without brain death. 770 97

Intracranial tumors are rare in the neonatal period, and generally the most common histological types are astrocytoma, medulloblastoma, choroid plexus papilloma and neuroectodermal tumors. The early diagnosis of these tumors is often very difficult. The authors report a case of a full-term newborn who presented with opisthotonus. A subependymal mass was detected by cerebral ultrasonography, and when the child was 1 month of age depigmentations appeared on the trunk and on the right leg, confirming the suspicion of tuberous sclerosis. At 3 months of age the child suffered infantile spasm with hypsarrhythmia. The developmental delay, the marked progressive neurological deterioration and the daily seizures suggested surgical resection. Histologic studies showed a subependymal giant cell astrocytoma such as typically occurs in tuberous sclerosis.
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PMID:Neonatal diagnosis of tuberous sclerosis. 867 81

We report a case of multiple parietal cavernous malformations in a thirteen year old female who received cranial irradiation following incomplete resection of a choroid plexus papilloma. The cavernous malformations, which developed within the prior parietal radiation ports, were diagnosed nine years after the patients' radiation treatment when the patient presented with increasing frequency of seizures. Family history was negative for familial cavernous malformations. Due to the worsening frequency of seizures, the patient underwent resection of these two cavernous malformations with diagnosis confirmed by pathology. Post-operative, there was a significant reduction in seizure frequency. The origin and pathophysiology of cavernous malformations remains controversial. Cranial radiation treatment for tumors, particularly in children, may possibly lead to the development of these lesions, as occurred in this case. This is, to our knowledge, the first case of multiple cavernous malformations occurring within a previous radiation field following radiotherapy for a neoplasm.
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PMID:Bilateral cavernous malformations resulting from cranial irradiation of a choroid plexus papilloma. 971 44

The nevus sebaceus syndrome (NSS) is a neurocutaneous disorder characterized by unilateral hyperplasia of skin appendages and skeletal hemihypertrophy, hemimegalencephaly, or hemiatrophy along with disabling seizures. Despite the proneness of the dermal stigmata to eventually undergo neoplastic transformation, the malformative lesions of the central nervous system rarely evolve into frank tumors. We present the case of a 10-year-old girl with left-sided sebaceus nevi, ipsilateral enlargement of the skull, and a desmoplastic neuroepithelial tumor (DNET) in the right fronto-parietal area of the brain. The tumor was removed by surgery. Histologically, it corresponded to a mitotically active small-cell anaplastic astrocytoma with genuine desmoplasia. Investigative methods included immunohistochemical positivity for glial fibrillary acidic protein, lack of expression of neuronal markers, and ultrastructural documentation of sheaths of basal lamina and collagen around tumor cells. A survey of the literature of brain tumors associated with NSS revealed two cases of histologically verified pilocytic astrocytomas, and one each of a choroid plexus papilloma, a mixed glioma, and a meningioma, as well as a subependymal giant cell astrocytoma--the latter possibly in an overlap syndrome of NSS and tuberous sclerosis. We hypothesize that the tumor described herein, one involving both atypical differentiation and enhanced growth potential, is paradigmatic of neuropathological events to be expected in the NSS.
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PMID:Desmoplastic neuroepithelial tumor of infancy in the nevus sebaceus syndrome: report of a unique constellation and review of the literature. 1135 15

Three neoplastic lesions located in the temporal horn of the lateral ventricle were diagnosed after the onset of seizures. Neither hydrocephalus nor temporal horn entrapment were present. The complete surgical removal of the tumor resulted in the disappearance of the epileptic episodes in 2 cases; the third patient refused surgery, and his lesion appears unmodified after a 3-year follow-up. Histological examination led to a diagnosis of choroid plexus papilloma in 1 case and of neurocytoma in the other; in the third case, the nonextracted lesion was presumed to be a partially calcified choroid plexus papilloma. The mechanism underlying the seizures in the 2 operated children is speculated to be a compression exerted upon the mesial temporal structures that form the medial wall of the temporal horn of the lateral ventricles; this compressive activity was resolved by removal of the tumor, and the epileptic episodes consequently disappeared in the 2 operated children; seizures were controlled by drugs in the third child.
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PMID:Tumors in the temporal horn of the lateral ventricle as a cause of epilepsy. 1807 15


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