IgG4-related sclerosing pachymeningitis
: a previously unrecognized form of central nervous system involvement in IgG4-related sclerosing disease.
PCR for Mycobacterium tuberculosis DNA on CSF has been reported positive infrequently in localized cranial pachymeningitis
denoting that it cannot be relied upon to exclude diagnosis of TB.
(4) Some--those with aortitis, retroperitoneal fibrosis, proximal biliary strictures, hypertrophic pachymeningitis
, pancreatic enlargement, or pericarditis--require treatment urgently to prevent irreversible disease-related complications or death.
Suzuki, "A case of definite IgG4-related pachymeningitis
," Neurology, vol.
in granulomatosis with polyangiitis: case series with earlier onset in younger patients and literature review," Clinical Rheumatology, vol.
Abbreviations: LPM- Lymphoplasmacyte- rich meningioma; WHO- World Health Organisation; IHP- idiopathic hypertrophic pachymeningitis
; EMA- Epithelial membrane antigen
In 1929 article we can find this statement: "Thirty-five years ago von- Bechterew wrote the first of a series of papers which described a syndrome consisting of stiff spine, nerve root pains, nerve root degeneration, alterations of sensation and muscle atrophies of a radicular distribution, which he ascribed to a pachymeningitis
and compression of the nerve roots.
Also have pachymeningitis
cervicalis (compression of the cervical cord secondary to glycosaminoglycans accumulation in the dura), claw-hand deformity, genu valgum, painful feet and pes cavus.
One patient had retrosellar extension, brain stem compression, and hydrocephalus with raised pressure manifestations (right third nerve palsy and right upper motor neuron); he also had pachymeningitis
Hypertrophic cranial pachymeningitis
is a rare chronic fibrosing inflammatory disease characterised by localized or diffuse thickening of duramater, leptomeninges, and tentorium.