Clinico-radiological spectrum and outcome in idiopathic hypertrophic pachymeningitis

Objective: To elucidate the clinico-radiological features, treatment response and outcome of a large cohort of patients (n = 20) with idiopathic hypertrophic pachymeningitis (IHP) and to examine if any of these features could differentiate between IHP and secondary causes of hypertrophic pachymeningitis (SHP). Methods: 20 patients with IHP diagnosed between 1998 and 2009 formed the study cohort. We adopted a validated clinical score to quantitatively assess and document their neurological disability and to compare their pre- and post-treatment outcomes. Appropriate statistical analysis was done to look for any clinical and/or radiological features to differentiate IHP from SHP. Results: Out of the twenty eight consecutive patients with pachymeningitis, 20 were having IHP and 8 were having SHP (Tuberculosis-5, Sarcoidosis-2, Wegener's granulomatosis-1). In IHP, headache and visual symptoms dominated the clinical symptomatology (80% and 75%). In MRI, the peripheral pattern of contrast enhancement was more common with IHP (p = 0.03). The posterior falx and tentorium showing a hypointense center ("fibrosis") and enhancing periphery ("active inflammation") together mimicking "Eiffel-by-night" sign was found to be more commonly associated with IHP (60% vs 12.5%, p = 0.03). Biopsy was done in 9 patients. At a mean follow-up of 51 months (range 24-144 months), the mean pretreatment clinical score improved from 6.55 to 1.80 in 20 patients with IHP (p < 0.001). Conclusions: Our data on the largest cohort of patients with IHP would shed light into its dinico-radiological spectrum, treatment and outcome. The prognosis is satisfactory if managed appropriately. We have highlighted the role of MRI in differentiating between IHP and other causes of SHP. (C) 2015 Published by Elsevier B.V.