Right Superior Oblique Paralysis associated with Idopathic Hypertropic Cranial Pachymeningitis

PURPOSE: Idiopathic hypertrophic cranial pachymeningitis (IHCP) is a rare disase, which causes chronic progressive inflammation and thickening of the basal dura mater. We report a case of IHCP associated with superior oblique paralysis and present a review of the literature. METHODS: An 8-year-old boy presented with binocular diplopia and left side head tilting. Suspecting right superior oblique muscle paralysis, an alternating prism test, head tilt test, fundus examinations, neurologic examination, and brain MRI were performed. RESULTS: The brain MRI revealed abnormal enhancement of the right tentorium in the course of the right fourth cranial nerve, leading to a diagnosis of IHCP with paralytic strabismus and the patient was treated with systemic steroid therapy. CONCLUSIONS: We report a case of IHCP with right superior oblique paralysis. Patients with recent onset paralytic strabismus require appropriate neurolgic and neuroimaging examinations.

A Case of Idiopathic Hypertrophic Cranial Pachymeningitis Associated with Tolosa-Hunt Syndrome

Idiopathic hypertrophic cranial pachymeningitis (IHCP) is a rare disease, which causes chronic progressive inflammation and thickening of the basal dura mater and produces multiple cranial neuropathies, headache, ataxia, and seizure. A 40 year-old man presented with a continuous, diffuse, dull headache with left periorbital pain, exophthalmos, ptosis and loss of visual perception. Brain MRI revealed thickened, highly enhanced pachymeninges in all supratentorial areas in association with the left cavernous sinus. A subsequent meningeal biopsy showed non-specific chronic inflammations of the pachymeninges. The patient dramatically responded to steroid therapy. To our knowledge, this is an extremely rare case of IHCP associated with Tolosa-Hunt syndrome. (J Korean Neurol Assoc 19(1):56~59, 2001

Idiopathic hypertrophic cranial pachymeningitis: Report of 2 cases & Review of literatures

Idiopathic hypertrophic cranial pachymeningitis(IHCP) is a rare chronic pro gressive fibrosing inflammation of pachymeninges of unknown origin. Since the spreading of CT and MRI, there has been a few cases of IHCP have been reported. We describe two patients of IHCP with brain parenchymal involvement presented as epilepsia partialis continua which has not been described as a symptom of IHCP and review the previous reported literatures. IHCP commonly presents headache, multiple cranial nerve palsy, ataxia, and sometimes seizure. In many cases, the CSF finding is noninfectious inflammation, and the erythrocyte sedimentation rate is elevated. The brain MRI is the best noninvasive tool for diagnosis of IHCP and shows diffuse thickening and enhancing dura, especially posterior fossa. IHCP responds steroid initially but recurs frequently and progresses chronically.