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Idiopathic Hypertrophic Pachymeningitis: A Report of Two Patients and Review of the Literature

  • Aaron S. Dumont (a1), Arthur W. Clark (a1) (a2), Robert J. Sevick (a1) (a3) (a4) and S. Terence Myles (a1) (a5)
Abstract
Purpose:

We report the treatment and follow-up, including MRI, of two patients with idiopathic hypertrophic pachymeningitis and review the English language literature, with emphasis on management and outcome in this rare disorder.

Methods and Materials:

The files of two patients were reviewed, with relevant histopathology and imaging (MRI). The first patient has been followed for sixteen years (the longest MRI-documented postoperative course reported for this condition) and the second for two years. The English language literature was reviewed, including a summary of all reported patients that have been followed with MRI or CT imaging.

Results:

Despite extensive investigation, no underlying etiology was determined in either patient. Histopathological studies revealed a chronic inflammatory dural infiltrate in both patients, with granulomas in the first but not the second patient. The first patient underwent surgery twice and has remained stable for sixteen years, despite persistent neurologic deficits. The second patient was managed with dexamethasone after a surgical biopsy, and experienced complete resolution of all neurological deficits and abnormalities seen with MRI.

Conclusion:

Although prompt and extensive surgery has been recommended for this condition, the results from our second patient indicate that complete remission can be achieved in some patients with biopsy and steroid therapy. This also supports the view that autoimmune mechanisms underlie idiopathic hypertrophic pachymeningitis. The first patient illustrates that extensive laminectomies may be an effective therapeutic option but chronic discomfort may result. If extensive surgery must be performed, laminoplasty should be done because of the potential for reduced pain and improved long-term spinal stability.

RÉSUMÉ: But:

Nous rapportons le traitement et le suivi, incluant la RMN, de deux patients ayant présenté une pachyméningite hypertrophique idiopathique et nous revoyons la littérature de langue anglaise en insistant sur le traitement et l'évolution de cette maladie rare.

Méthodes et sujets:

Les dossiers ainsi que l'anatomopathologie et l'imagerie (RMN) de deux patients ont été révisés. Le premier patient est suivi depuis seize ans (le suivi postopératoire le plus long documenté par RMN rapporté dans cette maladie) et le deuxième est suivi depuis deux ans. La littérature de langue anglaise a été révisée, incluant un sommaire de tous les cas rapportés qui ont été suivis par RMN ou CTscan.

Résultats:

Malgré une investigation poussée, aucune étiologie n'a pu être déterminée dans chacun de ces cas. Les études anatomopathologiques ont révélé une infiltration inflammatoire chronique de la dure-mère, avec des granulomes dans le premier cas seulement. Le premier patient a subi deux interventions chirurgicales et il est demeuré stable pendant seize ans, malgré des déficits neurologiques persistants. Le second patient a reçu de la dexaméthasone après la biopsie chirurgicale et a présenté une résolution complète de tous les déficits neurologiques et des anomalies observées à la RMN.

Conclusions:

Bien qu'on recommande de procéder rapidement à une chirurgie extensive dans cette affection, les résultats chez notre second cas indiquent qu'une rémission complète peut être obtenue chez certains cas par la biopsie et la corticothérapie. Ceci est en faveur de l'hypothèse d'un mécanisme autoimmun dans la pachyméningite hypertrophique idiopathique. Le premier cas illustre que les laminectomies extensives peuvent être une option thérapeutique efficace, mais qu'un inconfort chronique peut s'en suivre. Si une chirurgie extensive doit être effectuée, une laminoplastie devrait être faite pour minimiser la douleur et assurer une meilleure stabilité de la colonne vertébrale à long terme.

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Canadian Journal of Neurological Sciences
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  • EISSN: 2057-0155
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