Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018681 (headache)
 56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Sinus histiocytosis or Rosai-Dorfman disease (RDD) is a rare idiopathic histioproliferative disorder typically characterized by painless cervical lymphadenopathy, fever, and weight loss. Extranodal, intracranial disease is uncommon. In this report the authors describe the first case of intracranial RDD treated with stereotactic radiosurgery after resection. This 52-year-old man with known RDD presented with a 7-day course of fever, headache, diplopia, left facial paresthesias, and difficulty swallowing. No cranial nerve deficits were evident on examination, but right submandibular and inguinal node enlargements were noted. On neuroimaging, the patient was found to have a homogeneously contrast-enhancing petroclival lesion with extension into the left cavernous sinus. The patient underwent a combined left petrosal craniotomy and partial labyrinthectomy with duraplasty for biopsy sampling and partial microsurgical resection of the lesion. Microscopic examination of the biopsy specimen revealed the presence of a mixed cellular population with predominant mature histiocytes consistent with RDD. The residual tumor was treated with stereotactic radiosurgery 2 months after resection. On follow-up imaging the lesion had regressed significantly, with only slight dural enhancement remaining. Microsurgical resection for histological diagnosis, followed by stereotactic radiosurgery for residual tumor represents one treatment alternative in the management of intracranial RDD in which a complete resection carries the potential for excess morbidity.
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PMID:Intracranial Rosai-Dorfman disease treated with microsurgical resection and stereotactic radiosurgery. Case report. 1254 66

The clinical, radiological, and surgical issues concerning cavernous hemangiomas located within the dural confines of the cavernous sinus were analyzed on the basis of experience with 13 cases. The feasibility of radical resection by an entirely extradural approach using a basal temporal surgical route to this relatively rare and formidable surgical problem was investigated. Thirteen patients, four males and nine females, with cavernous hemangioma involving the cavernous sinus were treated from 1992 to 2001. The patients were aged from 15 to 55 years. Headaches and deficits of the cranial nerves coursing through the cavernous sinus were the principal symptoms at presentation. Vision was affected in four patients. The radiological features in all patients were similar with a characteristic pattern of extension and encasement of internal carotid artery. The maximum size of the tumor was 28 to 73 mm (mean 44 mm). An entirely extradural route using the basal temporal approach was used successfully in seven cases. Total resection was achieved in 12 patients and partial resection was achieved in one patient. The follow up ranged from 8 months to 9 years (mean 45 months). The outcome of extraocular movements was poor in our series, possibly due to the massive sizes of the tumors encountered. There was no recurrence or growth of the residual tumor and all patients were leading active lives.
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PMID:Extradural approach for cavernous hemangioma of the cavernous sinus: experience with 13 cases. 1269 17

We report a Taiwanese boy who presented with apoplexy of a prolactinoma. A 12 9/12 year-old boy presented to our clinic with headache and visual deficit of bitemporal hemianopsia. Skull X-ray showed an enlarged sella. Magnetic resonance imaging (MRI) of the sella turcica showed a 4 x 2.5 x 2.5 cm mass, located at the sella turcica and extending upward to compress the optic chiasm. Preoperative laboratory data showed hyperprolactinemia, hypothyroidism and hypocortisonism. After a stress dose of i.v. hydrocortisone was given, he underwent transsphenoid surgery to remove the tumor. Immunohistochemical stains were positive for PRL in the tumor cells. After surgery, he suffered from neurogenic diabetes insipidus, hypopituitarism and hyperprolactinemia, with serum PRL level of 491 ng/ml. Visual field examination was normal 4 months later. In conclusion, pituitary apoplexy is rare in children but should be considered if a patient suffers from headache, vomiting, and visual deficit. Brain MRI is preferred for diagnosis. Dopaminergic agonists should be given if residual tumor or recurrence of prolactinoma is found after transsphenoidal surgery.
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PMID:Pituitary apoplexy due to prolactinoma in a Taiwanese boy: patient report and review of the literature. 1471 55

Craniopharyngiomas are rare brain tumors of the hypothalamo-pituitary region, developing from embryonic remnants of Rathke's pouch and sac. Their overall incidence is 0.13 per 100,000 person years. Most frequently, they are suprasellar, start growing in childhood and originate neurological and hormonal symptoms. We retrospectively studied patients treated in our institution for craniopharyngioma in the last 10 years, in order to evaluate their clinical, imaging and pathological characteristics. Of the 32 patients analysed, 18 were females and 14 males with ages ranging between 6 and 81 years (early onset group--EOG aged 5-14 years: 7 patients; middle age onset group--MAOG aged 15-49 years: 15 patients; late age onset group--LOG aged > or = 50 years: 10 patients). Visual impairment was the most frequent presenting clinical feature in EOG (71.4%) and MAOG (86.6%), while in the LOG personality and cognitive changes including memory loss predominated (60%). Headaches were very frequent in all groups (EOG 42.8%, MAOG 60%, LOG 40%). Meningitis and seizures were presenting features, each in one patient. Regarding endocrine symptoms and signs, growth failure was present in 57.2% of the EOG. Amenorrhea was present in 5 of 10 female patients of the MAOG. Preoperatively, TSH was deficient in 25%, ACTH in 15.6% and gonadotropin in 25% of the patients. There were no cases of diabetes insipidus. Preoperative CT and MR revealed a calcified mass in 12 (37.5%), a partially cystic mass in 20 (62.5%) and a lesion involving or extending into the third ventricle in 7 (21.9%) patients. Twenty seven (84.4%) patients were treated primarily by surgery. In 4 (12.5%) cases the tumour was considered inoperable and 1 (3.1%) patient refused surgery; all were in the LOG. Surgical approach was transsphenoidal in 2/27 (7.4%) (all of them in the LAOG) and by craniotomy in the others. The tumour removable was considered complete in 10 (37%--EOG 2/7, MAOG 6/15, LOG 2/5) and subtotal in 17 (62.9%) patients. Eight (29.6%) patients were reoperated for recurrent tumour. Postoperative radiotherapy was administered in 12 cases with residual tumor, and 3 inoperable tumors were treated primarily by conventional external radiotherapy. Pathological study revealed the adamantinomatous type in 25 (92.6%) and the papillary type in 2 (7.4%--all men in the MAOG) tumors. The average follow-up was longer in the EOG (82.6 +/- 40.7 months) than in MAOG (57.2 +/- 48.5 months) and in LOG (48 +/- 92 months). Four (12.5%) patients died, 1 during the follow-up period due to a radiation-induced astrocytoma and 3 in the postoperative period because of cerebral hemorrhage and hydrocephalus (1 in the EOG and 2 in the LOG). In summary, we found the clinical presentation to be different in the 3 age groups, with a large number of patients in the MAOG. In this group were the only examples of the papillary form. Better prognosis was associated with a total resection at initial surgery.
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PMID:[Craniopharyngiomas. Clinicopathological aspects in different age groups]. 1552 57

A 3-year-old boy presented with headaches, vomiting, lethargy and papilledema. Communicating hydrocephalus along with transependymal fluid absorption and meningeal contrast enhancement was identified on CT. The enhancement was initially thought to be the result of a partially treated meningitis (child was previously on oral antibiotics for a presumed mycoplasma pneumonia). A right ventricular-peritoneal shunt was placed. CSF studies procured during the procedure were all normal. In contrast, CSF from a lumbar puncture contained a high protein, and cytology was highly suspicious for malignancy. Spine MRI showed diffuse leptomeningeal enhancement and a 1.5-cm intramedullary lesion at T12-L1 associated with minimal edema. The lesion was subtotally resected (70%) and diagnosed as an astrocytoma (mostly Kernohan grade 2 but with areas of grade 3). Chemotherapy was administered and follow-up spine MRI at 2 months did not reveal any residual tumor, however, the leptomeningeal enhancement persisted. Sixteen months later, at the completion of the chemotherapy and radiation therapy, the spine MRI remained unchanged. Neurological examination has always been normal. This case illustrates how a spinal cord astrocytoma can metastasize via spinocranial dispersion and present early with hydrocephalus rather than myelopathy.
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PMID:Hydrocephalus as the initial presentation of a spinal cord astrocytoma associated with leptomeningeal spread. 1588 10

A 34-year-old man presented with progressive diminution of vision in the left eye for 7 years. He had suffered left hemicranial headache associated with left retro-orbital pain and diplopia for 3 months. Magnetic resonance (MR) imaging revealed a pituitary tumor located in the sella and extending into the right cavernous sinus. After transsphenoidal surgery, the vision improved drastically but the diplopia persisted. Postoperative MR imaging showed residual tumor in the right cavernous sinus. Follow-up examination after 3 years showed the diplopia had completely recovered and the residual tumor in the cavernous sinus had disappeared. Spontaneous resolution of a large intracavernous sinus residue of a pituitary adenoma may occur due to tumor necrosis.
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PMID:Spontaneous resolution of residual pituitary adenoma. Case report. 1597 66

We report the case of a macroprolactinoma in a 32-year-old woman, who presented with secondary amenorrhea, galactorrhea, increased plasma prolactin level (3259 ng/ml), headache and bi-temporal visual field defect. Magnetic resonance imaging showed a large pituitary tumor. The patient responded well to bromocriptine (7.5 mg/day) with improvement of clinical symptoms and normalization of plasma prolactin within a few weeks. After 4 months of treatment, tumor size was also reduced markedly. During continued treatment at the same dose of bromocriptine the plasma prolactin level remained normal, but after 8 months of treatment the patient suddenly complained of worsening of her visual fields, and magnetic resonance imaging indicated re-enlargement of the tumor. Bromocriptine was discontinued and transsphenoidal pituitary surgery was performed. After surgery the visual field defect improved, but postoperative plasma prolactin level (1104 ng/ml) and magnetic resonance imaging indicated a residual tumor. Postoperative treatment with quinagolide (0.15 mg/day) resulted in disappearance of all clinical symptoms, normalization of prolactin level and a reduction in size of the residual tumor. This case demonstrates that a dissociation of the inhibitory effect of bromocriptine on tumor size and prolactin level may rarely develop during the course of drug treatment in a patient with macroprolactinoma.
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PMID:Rapid re-enlargement of a macroprolactinoma after initial shrinkage in a young woman treated with bromocriptine. 1601 80

Radiation induced meningiomas (RIM) are rare late complications in patients who have received high dose irradiation for brain tumors. The mean latency period for induction of RIM in most of the series is 18.7+/-10.2 years. There are only 9 reported cases of RIM following high dose cranial irradiation with unusually short latency periods of less than 5 years. Herein, we report a child diagnosed with RIM with an unusually short latency period of 14 months. An 11-year old male child underwent gross total resection of medulloblastoma. Following surgery he received high dose craniospinal irradiation. Postoperative computed tomography scan (CT scan) after 1 month did not show features of any residual tumor, recurrence or tumor at a new site. The child was asymptomatic for 14 months and then presented with complaints of headache and vomiting. CT scan head showed multiple solid homogenously enhancing lesions in bilateral basifrontal and right basitemporal region. Histopathology of the lesions turned out to be atypical meningioma.
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PMID:Radiation induced meningioma with a short latent period following high dose cranial irradiation - case report and literature review. 1629 89

This 50-year-old woman presented with headache and visual disturbance. Neuroimaging results demonstrated a well-demarcated tumor attached to the falx cerebri near the transverse sinus, and the lesion was subtotally removed. Based on histological and immunohistochemical evaluation, a solitary fibrous tumor (SFT) was diagnosed. During the next 32 months, the size of the residual tumor increased slightly at the transverse sinus. Stereotactic radiosurgery (SRS) was performed as an adjuvant treatment. Over the course of the next 4 years the tumor decreased in size. The authors suggest that SRS constitutes good adjuvant treatment for regrowing residual SFTs not amenable to reoperation.
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PMID:Stereotactic radiosurgery as adjuvant treatment for residual solitary fibrous tumor. Case report. 1712 Nov 44

We report a rare skull base neurocytoma. A 44-year-old female with a history of focal seizure and progressive right-sided weakness sought treatment at an outside institution, where she underwent total resection of a "left medial sphenoid wing paraganglioma" in 1984. In 1995 after experiencing intense left-sided headaches for 3 weeks, the patient presented to our institution. Magnetic resonance imaging revealed a large local recurrence. She had deficits dating to her initial surgery, including moderate right-sided hemiparesis, complete left ophthalmoplegia, and left facial numbness.The patient underwent a craniotomy with extensive removal of the involved sphenoid bone and a subtotal resection of the tumor. Neurocytoma was diagnosed based on strong immunohistochemical staining for synaptophysin and no reactivity for glial fibrillary acidic protein. Postoperatively, her headaches resolved completely and her neurologic status remained at baseline. The residual tumor was treated with radiation therapy. After 5 years, she remains clinically and radiographically stable.Although typically located adjacent to the foramen of Monro, neurocytomas have now been reported in almost every subcompartment of the craniospinal axis. Finding neurocytomas in extraventricular locations may require revisiting the current theory that subependymal progenitor cells are the cells of origin for these tumors.
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PMID:Skull base neurocytoma: case report and review of the literature of extraventricular neurocytomas. 1716 46


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