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Query: UMLS:C0018681 (headache)
 56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A unique association of Sturge-Weber syndrome and atlanto-occipital assimilation is presented. A 18-year-old male was admitted in emergency because of the sudden severe headache and vomiting. He had vascular nevus in the right half of the face at birth and several episodes of generalized convulsive seizures. On admission craniogram demonstrated calcification in the right occipital area. Neurological examination revealed tenderness in the nuchal region, moderate limitation of cervical mobility in a antero-posterior direction, Bruns-Cushing type nystagmus, bilaterally diminished gag reflex, and positive Romberg's test. Spinal tap showed crystal clear CSF with normal pressure. EEG showed paroxysmal slowing focus in the right parieto-occipital area. Polytomography of the craniovertebral junction demonstrated the unilateral atlanto-occipital assimilation on the left associated with the aplasia of the right posterior arch. Myelography was negative. A right carotid angiography disclosed the dilatation of the basal vein of Rosenthal and abnormal venous vasculature. CT-scan demonstrated the calcified region of the right occipital area more distinctly than the plain roentogenogram, but the enhancement study of the leptomeningeal angioma of the Sturge-Weber syndrome was negative. Never been found this rare association in a review of the literature, the authors discussed the clinical and radiological findings of both diseases.
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PMID:[Sturge-Weber syndrome associated with atlanto-occipital assimilation: a report of a case (author's transl)]. 68 66

Computed tomography scanning in two young patients with recurrent, pulsating, migraine-like headache showed parieto-occipital calcifications. One patient presented with an atypical form of the Sturge-Weber syndrome, and the other with celiac disease and folic acid deficiency. The clinical features were analyzed and compared with those in other cases reported in the recent literature which have shown bioccipital calcifications but no cutaneous angiomas, sometimes associated with visual and/or intelligence deficit and epilepsy. Finally, the possible connection between cerebral calcifications and headache is discussed.
Cephalalgia 1987 Jun
PMID:Bilateral cerebral occipital calcifications and migraine-like headache. 311 14

The patient was a 48-year-old housewife, who had a sudden onset of severe headache followed by loss of consciousness for a few hours on the day of admission. Initially she showed slight restlessness due to headache, neck stiffness and subhyaloid hemorrhage. Four-vessel study revealed a basilar aneurysm on right retrograde brachial angiography and anterior communicating aneurysm on left carotid angiography. Two weeks after the onset, when she had no neurological deficit except for intermittent appearance of disorientation, both aneurysms were successfully clipped through right pterional approach of Yasargil. The subarachnoid hemorrhage was apparently due to basilar bifurcation aneurysm. Postoperatively, she showed right hemiparesis including her face, aniscocoria (left, 4 mm, oval: right, 1.5 mm, round) and conjugate deviation toward the left. The disturbance of conjugate eye movement and the hemiparesis completely disappeared in 2 and 7 days respectively. The patient was discharged 4 weeks postoperatively with mild left 3rd nerve palsy. At present, one year postoperatively, she is fully engaged in her housewife life without any neurological deficits. A case of superior Foville syndrome combined with Weber syndrome after clipping of basilar bifurcation aneurysm was reported and its anatomicoclinical mechanism was reviewed. The pathogenesis was supposed to be left midbrain ischemic lesion due to circulatory disturbance of P-1 perforators (P-1: proximal posterior cerebral artery); e.g., occlusion on clipping of vasospasm. This P-1 perforator syndrome after aneurysmal clipping has been reported only little. The importance of preservation of these perforators with careful dissection and manipulation under microscopy was emphasized.
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PMID:[Superior Foville syndrome after clipping of basilar bifurcation aneurysm--case report (author's transl)]. 724 18

Based on reports of headache in arteriovenous malformations and epilepsy, one would predict that the prevalence of migraine headache in the Sturge-Weber syndrome would be higher than in the general population. A questionnaire designed to determine the prevalence and type of headache by IHS criteria was sent to the 500 members of the Sturge-Weber Foundation. The average age of the patients in this sample (71 patients) was 15 years. The sex ratio was approximately one to one. Headache meeting IHS criteria for migraine occurred in 28% (equally among males and females) compared to 17% in females and 5% in males in the general population. Neurologic deficits occurred in 58% during migraine. The prevalence of migraine in children under the age of 10 (31%) was higher than that reported in the general population (5%) and the age of onset was younger. The mechanism of migraine headache in Sturge-Weber syndrome may be similar to that found in epilepsy and vascular malformations.
Headache 1994 Oct
PMID:Headache in Sturge-Weber syndrome. 800 25

A patient with obstructive sleep-apnea-syndrome and congenital temporal and cervical hemangiomas developed severe headache under nasal BiPAP ventilation. Internal hydrocephalus was diagnosed by CCT and MRI and rise of intracranial pressure (ICP) under artificial ventilation was supposed. Polysomnography, ICP-measurement with a ventricular catheter and resistance to outflow-measurement were used to clarify differential diagnosis. During periods of apnea ICP was raised severely with a maximum in REM-sleep. With nasal ventilation no apnea was observed and ICP was normalized. Resistance to outflow was normal. A disturbance of CSF-dynamics or CSF-absorption leading to the patient's headache could be excluded. Duplex-sonography and cerebral angiography did not provide further information. After exclusion of other causes artificial respiration dependent swelling of the subcutaneous temporal and cervical hemangiomas was the most likely cause triggering the headache. A sufficient therapy however was not yet available. Probably the patient suffers from an abortive form of a Sturge-Weber-Krabbe-Dimitri-Syndrome.
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PMID:[Intracranial pressure in sleep apnea, hydrocephalus and congenital hemangioma. A case report]. 805 80

Sturge-Weber syndrome is characterized by the presence of a port-wine nevus, epilepsy, stroke-like episodes, headache, and developmental delay. We studied 20 cases to test the hypothesis that decreased cerebral blood flow alters neurologic function by affecting cellular glucose metabolism. Group A consisted of 10 patients with a mean age of 1.75 years and early seizure onset (6.8 months), whereas group B was composed of older patients (mean age, 15.3 years) with later onset of seizures (3.7 years). Neurologic disease was more severe in group A, but group B had more widespread structural brain defects - shown on computed tomographic scans and magnetic resonance imaging - and metabolic brain defects shown on hexamethylpropyleneamine oxime and [18F] fluorodeoxyglucose single photon emission computed tomographic scans. Six group A cases had hypoperfusion at baseline and five of nine had worsening of perfusion and glucose metabolism 1 year later. A total of 119 stroke-like episodes occurred in six group A cases and eight group B cases; there were 65% fewer strokes in children treated with aspirin. The data suggest that progressive hypoperfusion and glucose hypometabolism are associated with neurologic deterioration in Sturge-Weber syndrome. Longitudinal studies are needed to better define the natural history of disease and to evaluate the safety and efficacy of aspirin therapy.
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PMID:Central nervous system structure and function in Sturge-Weber syndrome: evidence of neurologic and radiologic progression. 988 31

We report a patient of atypical type of Sturge-Weber syndrome who demonstrated a reversible change by MRI FLAIR method in ictus and postictal state. A 5-year-old boy was admitted to our hospital because of severe headache, vomiting and loss of consciousness with his eyes conjugated to left for a few minutes. He had no facial nevus and other abnormal findings in physical examination. CT scan showed two small calcifications in the right occipital lobe. Postcontrast T 1-weighted image of MRI demonstrated a right parieto-occipital leptomeningeal enhancement. We diagnosed this case as an atypical type of Sturge-Weber syndrome. Although, on admission, FLAIR method showed the area of high signal intensity, after anticonvulsant therapy, those abnormal area disappeared. It is presumably detected by FLAIR method slight extravasation of plasma element in the surface of the brain due to regional hyperperfusion in ictus.
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PMID:[A case of atypical type of Sturge-Weber syndrome demonstrated reversible change by MRI FLAIR method in ictus and in postictal state]. 1045 52

With the exception of patients with neurofibromatosis type II, pediatric extraparenchymal cerebellopontine angle (CPA) tumors of any sort are extremely rare. Most gliomas encountered in the CPA in either children or adults involve the CPA as exophytic extensions of primary brain stem and/or cerebellar tumors. We encountered an unusual case of a giant CPA pilocytic astrocytoma arising from the proximal trigeminal nerve, completely separate from the brain stem. A nine-year-old girl with no evidence for any neurocutaneous syndrome, presented with headaches, mild obstructive hydrocephalus, trigeminal hypesthesia and a subtle peripheral facial paresis. Pre-operative neuroimaging suggested a petroclival meningioma. The tumor was completely resected via a right pre-sigmoid, retro-labyrinthine, subtemporal, transtentorial ('petrosal') approach, using intraoperative neurophysiological monitoring, with minimal morbidity. This appears to be the first reported case of a pediatric primary CPA glioma and the seventh reported case of primary CPA glioma, overall. It represents the second reported case of a primary CPA pilocytic astrocytoma. Given the findings in this case and the six other cases of primary CPA gliomas reported in the literature, as well as the results of histological studies of normal cranial nerves, we hypothesize that the point of origin of these rare and unusual tumors is the root entry zone of the involved cranial nerves. The differential diagnosis of primary CPA tumors should be expanded to include cranial nerve root entry zone primary CPA gliomas.
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PMID:Cranial nerve root entry zone primary cerebellopontine angle gliomas: a rare and poorly recognized subset of extraparenchymal tumors. 1121 99

Klippel-Trenaunay-Weber syndrome (KTWS) is a rare mesodermal phakomatosis characterized by (1) cutaneous haemangiomata (usually unilateral and involving an extremity) (2) venous varicosities and (3) osseous and soft tissue hypertrophy, also of the affected limb. Sturge-Weber Syndrome (SWS), also a mesodermal phakamatosis, is characterized by meningofacial angiomatosis with cerebral calcification. Overlap between KTWS and SWS is recognized. We describe the case of a young woman with features of both KTWS and SWS who presented with symptoms of acute intracranial hypertension, including headache, vomiting and marked visual impairment. Cerebral angiography revealed paucity of the superficial cortical veins overlying one of the cerebral hemispheres and centripetal venous drainage via small deep venous channels. Based upon this pattern of cerebral venous outflow, we postulate a mechanism to explain the acute episode of intracranial hypertension.
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PMID:Intracranial hypertension in Sturge-Weber/Klippel-Trenaunay-Weber overlap syndrome due to impairment of cerebral venous outflow. 1209 50

The Sturge-Weber syndrome was recently subdivided into type I (facial and leptomeningeal angioma, possible glaucoma), type II (facial angioma, without evident endocranial involvement), and type III (exclusive leptomeningeal angioma). Thus far in the literature only 24 cases of Sturge-Weber syndrome type III have been reported. This study presents a case of a 2-year 9-month-old child with normal psychomotor development and skin free (no angiomas), who presented repeated episodes of severe headache, vertiginous symptoms, vomiting, and drowsiness, separated by complete recovery. The cranial computed tomography and magnetic resonance imaging with gadolinium revealed left occipital leptomeningeal angiomatosis with calcifications, suggesting a diagnosis of Sturge-Weber syndrome type III. Considering the normal psychomotor development, the improved electroencephalographic reports between the episodes, and the absence of hypoperfusion areas on single-photon emission computed tomography at 30 months of follow-up, the symptomatology appears an expression of migraine-like symptoms resulting from vasomotor disturbances within and around the angioma, more than an expression of partial seizures arising through an epileptic focus in the ischemic region around the vascular malformation.
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PMID:Migraine-like attacks in child with Sturge-Weber syndrome without facial nevus. 1566 76


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