Artikel
IgG4-related hypertrophic pachymeningitis – a rare entity with elusive diagnosis
IgG4-assoziierte Pachymeningitis, eine seltene Entität mit schwer definierbarer Diagnose
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Autoren
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Majid Esmaeilzadeh
- Medizinische Hochschule Hannover, Neurochirurgie, Hannover, Deutschland
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Manolis Polemikos
- Medizinische Hochschule Hannover, Neurochirurgie, Hannover, Deutschland
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Mete Dadak
- medizinische Hochschule Hannover, Hannover, Deutschland
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Christian Hartmann
- medizinische Hochschule Hannover, Hannover, Deutschland
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Elvis J. Hermann
- Medizinische Hochschule Hannover, Neurochirurgie, Hannover, Deutschland
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Joachim K. Krauss
- Medizinische Hochschule Hannover, Neurochirurgie, Hannover, Deutschland
| Veröffentlicht: | 8. Mai 2019 |
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Gliederung
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Objective: IgG4-related hypertrophic pachymeningitis is a very rare disorder that may cause localized or diffused thickening of the dura mater. Misinterpretations of the clinical and imaging findings are common. Clinical manifestations depend upon the location of the inflammatory lesion and on compression of the nervous structures leading to functional deficits. A dural biopsy is commonly needed for a definitive diagnosis. Continuous medication with steroids, rituximab or methotrexate is considered the main treatment and is necessary to avoid disease recurrence.
Methods: Four patients with IgG4 related hypertrophic pachymeningitis were identified over a 4-years period. The parameters that we have considered in our cases include patient-related characteristics, the preoperative work-up of patients including signs and symptoms. Furthermore, the surgical treatment, fellow-up outcomes were analysed.
Results: There were two adults and two adolescents (mean age 32 years; range 15 to 67 years). Two patients were male, and two were female. Clinical symptoms leading to diagnosis were epilepsy (n=2), ataxia and nausea (n=1), and facial nerve palsy (n=1). MR imaging studies showed contrast enhancing lesions in the temporal region in 2 patients, and in the cerebellar region in the other 2 patients. Subtotal resection was performed in 2 patients and a biopsy via a suboccipital retrosigmoid approach was performed in the other 2 patients. Postoperative treatment consisted in the administration of glucocorticoids in 1 patient and of immunosuppressive agents in 3 patients. The postoperative course was unremarkable. There was no recurrence up to 24 months after surgery in 3 patients, while a progress was noted 4 years after surgery in one patient.
Conclusion: The diagnosis of IgG4 related hypertrophic pachymeningitis is challenging, but it may be safety achieved via craniotomy. More experience is needed to develop better treatment recommendations for management of IgG4-related hypertrophic pachymeningitis.
