cqvip:Background. Idiopathic hypertrophic cranial pachymeningitis (IHCP) is an unc ommon disorder due to localized or diffuse thickening of the dura mater. While h eadache is the most common manifestation, the clinical characteristics of the he adache in IHCP have not been well characterised. Methods. From 1996 to 2002, 6 consecutive patients with IHCP presenting with headache were reviewed (3 women, 3 men; mean age: 49 years). Diagnosis was based on characteristic neuroimaging findings and the exclusion of secondary causes of cranial pachymeningitis. This study reported the headache characteristics, neuroimaging features, and longitud inal follow up. Results. Chronic daily headache, especially chronic migraine, was the most common headache pattern observed (4/6 patients). Lateralization of headache location appeared to correlate with the distribution of the hypertroph ied durai lesions. Diagnostic delay was due to failure to evaluate with gadolini um enhanced MRI. After treatment, headache (5/6, 83%) and neurologic deficits (2/3, 67%) improved in most patients; however, followup MRIs (n = 5) showed det erioration in 3 patients. Conclusion. The headache of IHCP is typically a chro nic daily headache, often resembling chronic migraine. Correspondingly, IHCP sho uld be considered in the differential diagnosis of refractory chronic daily head ache, with or without associated cranial neuropathy or other associated neurolog ic deficits. The typical imaging finding on gadolinium enhanced MRI is localise d or diffuse pachymeningitis and failure to order a gadoliniumenhanced MRI is th e primary reason for delayed diagnosis. Despite symptomatic improvement on longi tudinal follow up, the MRI abnormalities may not improve in parallel with the c linical symptoms.
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