Hypertrophic pachymeningitis with sarcoidosis: a rare cause of craniocervical compression

Abstract

SUMMARYWe describe a case of a 58-year-old woman with a suspected dural tumour. She presented with progressive pyramidal weakness. MRI confirmed compression of the medulla oblongata and spinal cord at the level of C1-3. The localised dural mass lesion homogenously enhanced on T1 MRI and was considered most likely to be a meningioma. Incidentally, CT scan of the chest revealed peribronchial soft tissue thickening, suggestive of pulmonary sarcoidosis. Owing to the progressive nature of her weakness, she had a posterior occipitocervical decompression with a C1-3 laminectomy and resection of the thickened dura. Histology showed densely collagenous tissue with scanty psammoma bodies and multinucleate giant cells, consistent with hypertrophic pachymeningitis (HP)-a rare, chronic inflammatory condition, characterised by thickening and fibrosis of the dura. This case demonstrates that masses in the craniocervical junction can be varied in pathology and when there is evidence of systemic inflammation, HP should be considered. BACKGROUND

A case report of atypical long segmental thoracic hypertrophic pachymeningitis with ossification of ligamentum flavum and literature review

“…According to etiology, HP is classified as idiopathic and secondary type. Autoimmune disorders was identified as secondary causes of HP in previous reports, such as Rheumatoid arthritis [1], Multifocal fibro sclerosis [2], Wegener's granulomatosis [3], Neuro-Behçet's disease [4], sarcoidosis [5], ANCA related diseases [6][7][8], IgG4 related diseases [9][10][11][12]23] and antiphospholipid syndrome [13]. Some infectious diseases caused by bacteria [14,37], fungi [15] and Spirochete [16,17] has been reported associated with HP as well.…”

Differential diagnosis of hypertrophic pachymeningitis: literature review

“…Esant sar koi do zës su kel tam sme ge nø dan ga lø pa þei di mui, pas ta ra sis daþ niau siai lo ka li zuo ja si ties kau ko lës pa ma tu ir vir ðu ti ne nu ga ros sme ge nø da li mi. Dël ðios prie þas ties galû niø mo to ri kos ir ju ti mø su tri ki mai bei atak si ja yra daþnes ni nei esant ki tos etio lo gi jos HP [39,40]. Diag no zae patvir tin ti pa de da ki tø or ga nø sar koi do zës nu sta ty mas ir (ar) sme ge nø kie to jo dan ga lo biop si ja (pa pras tai ste bi mas granu lio mi nis ne nek ro ti zuo jan tis uþ de gi mas) [39,40].…”

“…Dël ðios prie þas ties galû niø mo to ri kos ir ju ti mø su tri ki mai bei atak si ja yra daþnes ni nei esant ki tos etio lo gi jos HP [39,40]. Diag no zae patvir tin ti pa de da ki tø or ga nø sar koi do zës nu sta ty mas ir (ar) sme ge nø kie to jo dan ga lo biop si ja (pa pras tai ste bi mas granu lio mi nis ne nek ro ti zuo jan tis uþ de gi mas) [39,40].…”