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Query: UMLS:C0018681 (headache)
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We presented a case of hemangioblastoma associated with spina bifida occulta, persistent metopic suture, thyroid adenocarcinoma, vertebro-occipital anastomosis and erythrocytosis. We have not found a hemangioblastoma with these associations, as far as we have seen in the literature. 36-year-old male was admitted with complaints of nausea, vomiting and ataxic gait in June, 1970. On admission, the examination revealed no evidence of increased intracranial pressure except for elevated CSF pressure by lumbar puncture and incoordination. The peripheral blood count disclosed slight erythrocythemia. Vertebral angiography revealed a vascular lesion of 2.0 cm in diameter situated almost in the midline of caudal cerebellum receiving its blood supply from the right posterior inferior cerebellar artery. In addition, a right vertebro-occipital anastomosis was visualized. Plain reoentgenograms showed persistent metopic suture and spina bifida occulta of C 5 - 6. After admission, installation of Ommaya reservoir and decompressive suboccipital craniectomy were performed, and a thyroid papillary adenocarcinoma was totally removed. After discharge, he had been well for two years until a month previously to the second admission, when he commenced to have again headache, nausea, and vomiting with ataxic gait. Vertebral angiography showed the tumor enlarged in size measuring 4.0 X 5.0 cm and the tumor stain was more irregular and less homogenous than 3 years before. Brain scan revealed an increased uptake in the midline of the posterior fossa. After readmission, in April, 1973, he gradually developed dysphagia, disturbance of articulation and inactivity of mentality and died from pneumonia in October, 1974. Autopsy revealed a vascular tumor originated from the medial portion of the right cerebellum and the tumor showed multiple cyst formation in the rostral part in contrast to the caudal solid mass. Histologically the tumor tissue was composed of capillaries supported by fine argyrophilic fibers, large clear interstitial cells containing lipid granules and hemosiderin pigment. Carcinoma of the right lobe of the thyroid was found with metastasis to the bone marrow, lungs and anterior cervical lymphnodes and lymphnodes at the left supraclavicular angle. Bone marrow showed marked erythropoiesis. The case reported here provides an evidence to suggest that there is more than a random relationship between hemangioblastoma, dysraphic state and thyroid carcinoma. The other association, the vertebrooccipital anastomosis may result from the enhanced demand of blood supply by hemangioblastoma but this speculation needs further examination.
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PMID:[A case of hemangioblastoma associated with spina bifida occulta, persistent metopic suture, thyroid adenocarcinoma, vertebro-occipital anastomosis and erythrocytosis (author's transl)]. 79 Feb 13

A case of a brain stem hemangioblastoma with recurrent episodes of hypacusis due to progression of hydrocephalus is reported. The patient was a 25-year-old female, admitted to the department of otorhinolaryngology with complaints of hearing difficulty, headache and blurred vision. Neuroradiological studies showed a tumor from the medulla oblongata, obliterating the IVth ventricle, and a secondary hydrocephalus. Hearing loss fluctuated as hydrocephalus progressed. Multiple V-P shunting procedures relieved episodic hypacusis. The patient remains asymptomatic at present and has resumed normal activity. The mechanism of episodic hearing loss due to hydrocephalus is though to be due to the fact that through the ductus perilymphaticus and the ductus endolymphaticus, especially the former, increased intracranial pressure is transmitted to the inner ear. Through the ductus perilymphaticus there is communication between the perilymphatic space and the intracranial subarachnoid space. Through the ductus endolymphaticus there is communication with the subdural space. Increased ICP effects the inner ear. It is suspected that, in this particular case, the progression of hydrocephalus effected the patient's hearing.
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PMID:[A case of hydrocephalus with hypacusis due to hemangioblastoma]. 204 55

A case is reported in which a diffuse subarachnoid dissemination occurred after successful removal of a posterior fossa hemangioblastoma. The patient was a 51-year-old man who suffered from headache and gait disturbance, and who was admitted to our hospital on September 25, 1985. Neurological examination revealed trunkal ataxia and increased intracranial pressure. A CT scan with contrast media revealed a high-density lesion with surrounding low-density area in the posterior fossa. Right vertebral angiography demonstrated a round tumor stain behind the medulla oblongata, which was fed by the right posterior inferior cerebellar artery. The complete resection of the tumor was carried out. A biopsy specimen from the tumor showed a picture characteristic of hemangioblastoma. Fourteen months later the patient was readmitted due to the sudden onset of disturbance of consciousness. A CT scan revealed a large intracerebral hematoma in the right putaminal region. Right carotid angiography demonstrated two small tumor stains in the right temporal lobe. An emergency operation to extirpate the hematoma was performed. But the disturbance of consciousness did not improve, and the patient died. At necropsy a lot of small round tumors were recognized at the surface of the bilateral cerebral and cerebellar cortex. They were present in the subarachnoid space and partially invaded the cerebral and cerebellar parenchyma, extending through Virchow-Robin's space. There was no recurrence of the tumor at the site of the original operation. No doubt, a subarachnoid dissemination had occurred. However the histological findings of tumors were similar to the findings recorded in the first biopsy.
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PMID:[A case of hemangioblastoma with subarachnoid dissemination]. 230 11

It has already been noted that hemangioblastoma is occasionally complicated with various diseases, especially retinal angioma, cysts of kidney and/or pancreas, vascular disorders, and furthermore about 10% of hemangioblastoma are multiple. The authors report here a case of multiple cerebellar hemangioblastomas accompanied with congenital deafness, juvenile diabetes mellitus and retinal angioma. The patient, a 38-year-old man who complained of disturbance of consciousness, headache and vomiting, admitted to our hospital on October 6, 1982. He was born in consanguineous marriage family and his elder sister was also suffered from congenital deafness and juvenile diabetes mellitus, but no angiomas. CT and angiography showed a left side cystic and a right side small cerebellar lesions with remarkable ventricular enlargement. Ventriculo-peritoneal shunt and suboccipital craniectomy & removal of the tumors were performed on October 6, and November 8, 1982 succeedingly. From a point of view of hereditary occurrence, the authors investigated these complicated lesions recognized in this patient. It revealed that this case did not belong to any other categories of hereditary syndromes which were already reported and so authors considered that this was an extremely rare condition. Moreover, in case of multiple hemangiomas, cerebral angiography could be a more useful method than CT scan to detect multiplicity of the lesions in the posterior fossa.
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PMID:[A case of multiple cerebellar hemangioblastomas with congenital deafness, juvenile diabetes mellitus and retinal angioma]. 370 51

Tumors of the posterior fossa presenting orthostatic hypotension are rare and only nine cases have been reported so far. The locations of almost all these tumors were near the fourth ventricle and three of them were hemangioblastoma. A case of a tumor of the fourth ventricle showing autonomic disturbances mainly composed of orthostatic hypotension is reported. A 42-year-old male was admitted to the Department of Neurology of Chiba University Hospital on June 25th, 1981 because of three years' history of autonomic disturbances including orthostatic syncope, impotence, urinary disturbance and bowel dysfunction such as vomiting, diarrhea and constipation. He also complained of weight loss and staggering of gait to the left side. On admission, the patient was emaciated being 50 kg in weight and 172 cm in height. Neurological examination revealed hippus of bilateral pupils in light reflex, saccadic eye movement, slightly hypoactive deep tendon reflexes, mild terminal oscillations in bilateral finger-to-nose test, oscillation in the left heel-to-knee test, staggering tendency of gait to the left, slightly impaired tactile and thermal sensations in distal parts of the legs. Autonomic disturbances were showed by orthostatic hypotension (BP 104-50 in supine and 70-40 in sitting position), impotence, weight loss, anorexia, decrease of sweating, spontaneous yawning and loss of sensation of bladder fullness. About 5 weeks after admission, he began to complain of temporal headache and showed impairment of memory, drowsiness, paroxysmal apnea and papilledema.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Progressive dysautonomia in hemangioblastoma in the region of the fourth ventricle]. 396 90

The patient was a 31-year-old female who complained of headache and gait disturbance and admitted to our hospital on January 14, 1983. Familiar history revealed that her mother died of hemorrhage from hemangioblastoma in area postrema. Neurological examination revealed bilateral papilledema, right cerebellar sign and ataxia. Other neurological and clinical examinations were normal. CT-scan showed a right cystic cerebellar lesion and a right intraventricular lesion suggestive of hemangioblastomas. Right vertebral angiography demonstrated two vascular tumors lying in the right cerebellar hemisphere and right trigone of the lateral ventricle. Operation was performed on January 28, 1983. The histological diagnosis were hemangioblastomas.
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PMID:[A case of concomitant posterior fossa and supratentorial hemangioblastomas]. 403 17

A 45-year-old male presented with sudden onset of severe headache, mild disorientation, and gait disturbance due to intracystic hemorrhage from a cerebellar hemangioblastoma. He was successfully treated with ventricular drainage followed by total removal of the tumor. Reported cases of intracranial hemangioblastoma with massive hemorrhage show male predominance, tend to be solid rather than cystic, and occur in supratentorial more than infratentorial locations. Thin-walled and dilated vessels in the present and other cases were a possible cause of hemorrhage. Massive hemorrhage from hemangioblastoma is rare but needs prompt treatment because of the relatively high mortality.
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PMID:Cerebellar hemangioblastoma with intracystic hemorrhage--case report. 747 91

Supratentorial hemangioblastoma is encountered very rarely. About 80 cases in all have been reported. The authors present a case of supratentorial hemangioblastoma in the thalamus with gradually enlarging cysts. The patient was a 45-year-old man with complaints of left hemiparesis and headache. Computed tomographic scans of the brain showed a cystic mass with mural nodule in the right thalamus. Left vertebral angiography demonstrated a vascular tumor fed by a thalamogeniculate artery. A right temporo-parietal craniotomy was performed and the tumor was totally resected through the transcortical approach. The histological diagnosis was hemangioblastoma. Post-operative course was good except for a minor complication of the left lower quadrant homonymous hemianopsia and left hemiparesis which had disappeared at the time of discharge.
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PMID:[A case of hemangioblastoma in the thalamus]. 781 83

Autonomic dysreflexia is a poorly understood entity, typically occurring in the spinal cord-injured patient, with paroxysmal hypertension, bradycardia, severe throbbing headache, anxiety and sweating above the level of the lesion. An 18-year-old man underwent removal of a hemangioblastoma from the inferior portion of the fourth ventricle, a region known as the area postrema. Postoperatively he exhibited signs of autonomic failure. He later developed recurrent paroxysmal episodes of abdominal pain, hypertension, skin flushing and headaches. He subsequently was found to have a gastric ulcer. Symptoms and signs significantly improved with its treatment. We postulate that diminished sympathetic outflow occurred as a result of the surgery, creating a situation similar to the spinal cord-injured patient. Autonomic dysreflexia was elicited as a consequence of the noxious input of the gastric ulcer. In other cases of brainstem tumor resection, unrecognized episodes of autonomic dysreflexia may occur. This case also indicates that sympathetic supraspinal control is located at the level of the medulla or higher.
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PMID:Autonomic dysreflexia after brainstem tumor resection. A case report. 826 Jan 35

The first documented case of a symptomatic intrasellar hemangioblastoma is described, occurring in an 11-year-old girl with stigmata of von Hippel-Lindau disease who presented with headaches, progressive bitemporal hemianopsia, and adenohypophysial dysfunction. A subtotal resection of the lesion was achieved with two separate surgical procedures: a transsphenoidal approach and a subfrontal craniotomy. Subsequent growth of residual tumor was treated with combined conventional radiotherapy and stereotactic radiosurgery. Two years following completion of these adjuvant therapies, no residual tumor was evident on magnetic resonance imaging. Previous experience with hemangioblastoma in this region, as well as the rationale for radiotherapy in the treatment of incompletely resected lesions, is reviewed.
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PMID:Symptomatic intrasellar hemangioblastoma in a child treated with subtotal resection and adjuvant radiosurgery. Case report. 884 70


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