BACKGROUND: Idiopathic Hypertrophic Pachymeningitis (IHP) is a rare chronic inflammatory disorder of the dura. Classic clinical symptoms include headaches and cranial neuropathy. Because of scarce clinical data from Thailand, the present study aimed to determine the clinical features, neuroimaging findings, natural histories, therapeutic options, and outcomes for treatment of IHP in a tertiary care center. MATERIAL AND METHOD: A retrospective study was carried out on all adult IHP patients hospitalized at King Chulalongkorn Memorial Hospital, Bangkok, Thailand, between January 2000 and November 2011. Diagnostic criteria included 1) clinical symptom compatibility with IHP, 2) neuroimaging to reveal enhanced hypertrophic dura compatible with clinical syndrome, and 3) ruled out secondary causes of LHP, using appropriate clinical profiles and investigations including tissue biopsy. RESULTS: Thirty-two patients were enrolled with 21 females and 11 males, mean age of 49.03 +/- 16.12 years. The two most common symptoms were headache (93.8%) and diplopia (43.8%). The most common neurological finding was multiple cranial neuropathies (84.4%). Cranial nerve III was affected in 56.3% of the patients, followed by other cranial nerves including CN VI, IV, V and II. Headache without a neurological deficit was observed in 12.5% of the cases. Focal and diffuse enhanced thickening of the dura were observed in 96.9% and 3.1% of the cases respectively. Focal thickening in the supratentorium included the cavernous sinus, orbital apex, sphenoid wing, and superior orbital fissure. Focal thickening in the infratentorium included the falx cerebelli, the dura at the base of the skull, Meckel's cave, and foramen magnum. CSF examination showed lymphocyte pleocytosis with a slight increase in CSF proteins. Headache subsided in all of the patients after treatment with corticosteroid In relapsing and recurrent patients, a combined treatment of steroids and azathioprine was prescribed. With the combined treatment, clinical complete recovery, relapsing and recurrence were detected in 40%, 40% and 20% of the cases respectively. All relapsing and recurrence were due to rapid tapering of for early discontinuation of the steroids treatment. Only one patient had a spontaneous remission. CONCLUSION: The most common clinical manifestations of IHP were headache and multiple cranial nerve involvement. Almost all of the patients had good initial response to steroid therapy. Relapse or recurrence was usually caused by rapid tapering off or early discontinuation of the steroid treatment. Long-term treatment with combined immunosuppression may be necessary in some cases.
BACKGROUND:Idiopathic Hypertrophic Pachymeningitis (IHP) is a rare chronic inflammatory disorder of the dura. Classic clinical symptoms include headaches and cranial neuropathy. Because of scarce clinical data from Thailand, the present study aimed to determine the clinical features, neuroimaging findings, natural histories, therapeutic options, and outcomes for treatment of IHP in a tertiary care center. MATERIAL AND METHOD: A retrospective study was carried out on all adult IHP patients hospitalized at King Chulalongkorn Memorial Hospital, Bangkok, Thailand, between January 2000 and November 2011. Diagnostic criteria included 1) clinical symptom compatibility with IHP, 2) neuroimaging to reveal enhanced hypertrophic dura compatible with clinical syndrome, and 3) ruled out secondary causes of LHP, using appropriate clinical profiles and investigations including tissue biopsy. RESULTS: Thirty-two patients were enrolled with 21 females and 11 males, mean age of 49.03 +/- 16.12 years. The two most common symptoms were headache (93.8%) and diplopia (43.8%). The most common neurological finding was multiple cranial neuropathies (84.4%). Cranial nerve III was affected in 56.3% of the patients, followed by other cranial nerves including CN VI, IV, V and II. Headache without a neurological deficit was observed in 12.5% of the cases. Focal and diffuse enhanced thickening of the dura were observed in 96.9% and 3.1% of the cases respectively. Focal thickening in the supratentorium included the cavernous sinus, orbital apex, sphenoid wing, and superior orbital fissure. Focal thickening in the infratentorium included the falx cerebelli, the dura at the base of the skull, Meckel's cave, and foramen magnum. CSF examination showed lymphocyte pleocytosis with a slight increase in CSF proteins. Headache subsided in all of the patients after treatment with corticosteroid In relapsing and recurrent patients, a combined treatment of steroids and azathioprine was prescribed. With the combined treatment, clinical complete recovery, relapsing and recurrence were detected in 40%, 40% and 20% of the cases respectively. All relapsing and recurrence were due to rapid tapering of for early discontinuation of the steroids treatment. Only one patient had a spontaneous remission. CONCLUSION: The most common clinical manifestations of IHP were headache and multiple cranial nerve involvement. Almost all of the patients had good initial response to steroid therapy. Relapse or recurrence was usually caused by rapid tapering off or early discontinuation of the steroid treatment. Long-term treatment with combined immunosuppression may be necessary in some cases.