|Gollwitzer M et al.|
Rezidivierende idiopathische hypertrophe spinale Pachymeningitis: Fallbericht // Recurrent idiopathic hypertrophic spinal pachymeningitis – case report
Journal für Neurologie, Neurochirurgie und Psychiatrie 2019; 20 (2): 72-74
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Keywords: Idiopathische Meningitis, Neurochirurgie, rezidivierende Pachymeningitis, Therapie, Wirbelsäule, idiopathic meningitis, recurrent pachymeningitis, spine, therapy
Idiopathic hypertrophic spinal pachymeningitis is a very rare chronic disease with frequently progressive and recurrent course manifesting itself as diffuse inflammatory dural fibrosis. Whereas acute treatment consists of immediate surgical decompression of the spinal cord in conjunction with steroid medication, the management of chronic and recurrent disease forms is controversially discussed in the current literature.
The patient described in this case report was admitted to our department with an acute onset of incomplete paraplegia. MR imaging disclosed a large, ventral, contrast enhancing, semicircular spinal mass extending from Th3 to Th9. Acute management included immediate spinal cord decompression by laminectomy and debulking of the mass lesion as well as subsequent medical treatment. The descriptive histopathologic diagnosis that of a chronic inflammatory process with granulation tissue formation in absence of signs of malignant, infectious or autoimmune diseases and idiopathic hypertrophic spinal pachymeningitis was established as diagnosis of exclusion. Under antibiotic and steroid medication, the paraparesis resolved almost completely. The further course of disease was complicated by recurrence of the spinal mass lesion, necessitating revision surgery 6 months after the initial operation. In close collaboration with the Department of Rheumatology, a long-term methotrexate and low dose steroid medication was subsequently established, resulting in clinical stabilization of the patient, i.e. independent ambulation, and in further regression of the spinal mass lesion as documented upon follow up MRI.
In view of the paucity of published experience with this rare disease process, the current body of literature lacks evidence based management guidelines. The case reports published so far have advocated different treatment algorithms which may be applied alone or in conjunction. In line with the sparse evidence available, we decided to establish a long-time medical treatment with Methotrexate and lowdose steroids, which proved both safe and effective in our hands.
Kurzfassung: Die idiopathische hypertrophe spinale Pachymeningitis stellt eine sehr seltene chronische Erkrankung dar, welche durch einen progredient rezidivierenden Verlauf einer diffus entzündlichen duralen Fibrose charakterisiert ist und letztendlich eine Ausschlussdiagnose ist. Während die Therapie im Akutfall in chirurgischer Rückenmarkdekompression in Kombination mit Steroidmedikation besteht, ist die Behandlung der chronisch rezidivierenden Verlaufsform umstritten.