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

We report seven patients with cranial pachymeningitis of unknown origin in whom the main clinical features were headaches, ataxia, and cranial nerve palsies. CSF showed inflammatory changes. CT and MRI showed thickening of the falx and of the tentorium. The clinical course was chronic. Four patients improved with prednisolone but became steroid-dependent: in two cases, radiotherapy had no lasting improvement and in one, azathioprine permitted a reduction of the corticosteroids. Five patients had biopsy of the tentorium cerebelli or of the temporal dura mater. In two cases, autopsy revealed extensive pachymeningitis without parenchymal changes. In all instances, microscopic examination of the dura mater showed a cellular infiltrate of polymorphic cells; there were no epithelioid granulomas. Review of the literature discloses seven similar cases. We discuss the relationship of these lesions with inflammatory meningeal masses, the focal pachymeningitis of the Tolosa-Hunt syndrome, and multifocal fibrosis.
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PMID:Cranial pachymeningitis of unknown origin: a study of seven cases. 832 33

Unexpected autopsy findings are presented of a patient who died suddenly after a 6-month history of progressive headaches, nausea, and vomiting. A ruptured Rathke's cleft cyst (RCC) was identified within the adenohypophysis, with a chronic inflammatory reaction in surrounding pituitary and overlying hypothalamus. A brisk lymphoplasmacytic response was also seen in the cavernous sinuses bilaterally, identical to the pathology reported for idiopathic painful ophthalmoplegia, also called Tolosa-Hunt syndrome (THS). The pathogenesis of THS has not been elucidated; based on the findings in this report, it is suggested that some THS cases may result from a hyperimmune response to RCC rupture with extension into one or both cavernous sinuses. Although prompt alleviation of symptoms with corticosteroid treatment is generally encountered with THS, recurrence of symptoms is not uncommon. A careful search for a ruptured RCC should be undertaken in atypical cases of THS, with possible consideration of surgical intervention.
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PMID:Ruptured Rathke's cleft cyst: a possible cause of Tolosa-Hunt syndrome. 910 Nov 12

Three cases of idiopathic hypertrophic cranial pachymeningitis are presented. The diagnosis was based on the CT scan or MRI findings (or both) of thickened enhancing dura. In all cases, meningeal biopsies were performed and microscopic findings were compatible with nonspecific inflammation. All cases presented with subacute and chronic localized headache. Two cases had associated chronic meningitis. One cases presented with a syndrome of multiple cranial nerve involvement (polyneuritis cranialis). Corticosteroids, in the form of prednisolone 60 mg/day, were effective in all cases. Two cases with less severe pachymeningitis received corticosteroids for 2 weeks, then were tapered off in 4 to 6 weeks. A case with extensive lesions needed a long-term low dosage of prednisolone, 5 to 10 mg/day for maintenance therapy. Idiopathic hypertrophic cranial pachymeningitis may be related to the Tolosa-Hunt syndrome, the syndrome of polyneuritis cranialis, and multifocal fibrosclerosis.
Headache 1997 Apr
PMID:Idiopathic hypertrophic cranial pachymeningitis: an unusual cause of subacute and chronic headache. 915 Jun 22

Two patients with retroorbital pain syndromes with or without paresis of cranial nerves developed weeks after ipsilateral headache resembling chronic paroxysmal hemicrania (CPH) but without autonomic features. These findings might support the hypothesis that CPH may be caused by a pathological process in the region of the cavernous sinus, as has been proposed for the Tolosa-Hunt syndrome (THS).
Cephalalgia 1997 Aug
PMID:A CPH-like picture in two patients with an orbitocavernous sinus syndrome. 925 79

The oculomotor nerve of a 6-year-old boy with recurrent headache and recurrent ophthalmoplegia was contrast-enhanced on a magnetic resonance imaging (MRI) scan during an episode. The boy exhibited dramatic response to steroid treatment. The clinical features of ophthalmoplegic migraine and Tolosa-Hunt syndrome overlapped in this patient. We suggest that a positive MRI finding can be included as one of the diagnostic criteria in the classification of ophthalmoplegic migraine and that a trial of steroid is worthwhile in the presence of enhancement of the oculomotor nerve since ophthalmoplegic migraine may be noninfectious but inflammatory in etiology.
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PMID:Enhancement of oculomotor nerve: a diagnostic criterion for ophthalmoplegic migraine? 930 81

The incidence, pathophysiology, symptoms, differential diagnosis and treatment of ophthalmoplegic and retinal migraines are reviewed. We describe three cases: one recurrent, painful ophthalmoplegia alternating between the III and VI nerves, one recurrent migraine associated with internal ophthalmoplegia, and one migraine with visual aura and retinal infarction. In our review we emphasize their infrequency (ophthalmoplegic migraine, 0.7 per million; retinal migraine 0.5-7% of migraines with aura), their uncertain pathophysiology and the possibility of causing permanent oculomotor or visual deficits. Differential diagnoses, including all causes of painful ophthalmoplegia and amaurosis fugax are discussed, with special attention to the difficulty of differentiating Tolosa-Hunt syndrome and forms without headache.
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PMID:[Ophthalmoplegic and retinal migraines]. 964 38

A 23-year-old woman was admitted with headache, nausea, vomiting and blurred vision on the left side. Neurological examination showed ptosis with a complete internal and external ophthalmoplegia and a red fullness around the left orbita. Computed tomographic scanning of the brain revealed no abnormalities. As she improved on high doses of steroids a diagnosis of Tolosa-Hunt syndrome (THS) seemed to be indicated. However, magnetic resonance imaging (MRI) showed a lesion with intermediate signal intensity in the left cavernous sinus. Craniotomy was performed when symptoms of THS recurred. Histopathological examination revealed a meningioma with a papillary aspect and some mitoses. This case illustrates that: (1) THS is still a diagnosis by exclusion; (2) MRI and histopathological examination are important if there is any doubt about the diagnosis; and (3) also when there is no doubt, improvement after steroid therapy may be a diagnostic pitfall. Therefore, not only MRI but also orbital phlebography and angiography should seriously be considered.
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PMID:Meningioma presenting as Tolosa-Hunt syndrome. 1035 Jan 98

In 1988 the International Headache Society defined the diagnostic criteria of Tolosa-Hunt syndrome (THS) to include episode(s) of unilateral orbital pain for an average of 8 weeks if untreated, with associated paresis of one or more of the third, fourth, and sixth cranial nerves. Cranial nerve paresis may coincide with the onset of pain or follow it within a period of up to 2 weeks, and the pain must be relieved within 72 h after the initiation of corticosteroid therapy. Other causative lesions must be excluded by neuroimaging. On the basis of the history and neuroradiological findings of six patients we show the pitfalls in diagnosing THS with these criteria. We propose a revision of the criteria: Other causative lesions must be excluded by neuroimaging, especially of the region of the cavernous sinus and the orbita, and by blood and CSF examinations. Since imaging techniques have dramatically improved, it is now possible to visualize the inflammatory tissue in THS. Positive magnetic resonance imaging or computed tomography findings compatible with inflammatory tissue neither exclude nor confirm THS and remain suspect until a malignant tumor or inflammation other than THS is excluded. Clinical and radiological follow-up examinations must be performed for at least 2 years, even in patients with negative findings on magnetic resonance imaging at onset.
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PMID:The criteria of the International Headache Society for Tolosa-Hunt syndrome need to be revised. 1039 69

Ten patients (6F, 4M) with recurrent Tolosa-Hunt syndrome are reported. Besides ocular motor symptoms, one patient had trigeminal nerve involvement, one had ipsilateral ocular sympathicoplegia with miosis and ptosis, and one tinnitus during an episode of Tolosa-Hunt syndrome, ipsilateral to the pain side. One patient had Bell's palsy, one had a possible Raeder's syndrome, and one had a period of tinnitus between the Tolosa-Hunt syndrome episodes. Three of the 10 patients reported periods of periocular pain without ophthalmoplegia between the Tolosa-Hunt episodes, the pain located ipsilateral to the ophthalmoplegic side in the Tolosa-Hunt episodes. Systemic symptoms associated with Tolosa-Hunt syndrome, e.g., back pain, chronic fatigue, arthralgia, gut problems among others, occurred with the same frequency in these 10 patients as in an earlier report. Seventy per cent of the patients had signs of inflammation in serum during a period of Tolosa-Hunt syndrome. Orbital phlebograms showed pathologic signs in four of the five patients investigated during a Tolosa-Hunt period. One phlebogram was normal in a sixth patient when performed during a period of unilateral periocular pain without ophthalmolegia. Magnetic resonance imaging of the head (with contrast) was only performed in three patients during the Tolosa-Hunt period: one showed signs of inflammation in the middle fossa and two were normal. In one of the patients with normal magnetic resonance imaging, the orbital phlebogram was pathologic. Steroid treatment promptly relieved the pain in all patients.
Cephalalgia 1999 Dec
PMID:Recurrent Tolosa-Hunt syndrome: a report of ten new cases. 1066 17

Experience with modern neuroimaging techniques, computed tomography (CT) and magnetic resonance imaging (MRI) scans, in the diagnosis of Tolosa-Hunt syndrome (THS) is reviewed. Conventional CT scan remains normal in about two-thirds of these patients. In the reported 22 patients meeting the IHS criteria for a THS diagnosis on whom an MRI study was performed, MRI revealed a convex enlargement of the symptomatic cavernous sinus by an abnormal tissue isointense with gray matter on short TR/TE images and isohypointense on long TR/TE images. This abnormal tissue markedly increases in signal intensity after contrast injection. MRI seems also to be the ideal technique to follow progressive resolution of the abnormal tissue after steroids. Therefore, normal MRI would probably exclude THS, whereas in the appropriate clinical setting of steroid-responsive painful ophthalmoplegia, MRI showing the cavernous sinus abnormality described here suggests a diagnosis of THS. From these data, we propose that the fourth IHS criterion for THS diagnosis, "Exclusion of other causative lesions by neuroimaging and (not compulsory) carotid angiogram" should be changed to "Finding by MRI of specific cavernous sinus abnormalities (with the characteristics described herein) which slowly resolve with steroid treatment".
Cephalalgia 1999 Dec
PMID:Tolosa-Hunt syndrome: focus on MRI diagnosis. 1066 18


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