IgG4 associated Hypertrophic Pachymeningitis: A rare entity

in Max Super Speciality Hospital, Shalimar Bagh

Jan 16 , 2023

A 53-year-old woman came with with left-sided hemicranial headaches, dull ache, continuous, intermittently pricking pain on the left side orbital region associated nausea, photophobia, and phonophobia for 5 months; increased since 2 days also on the right-sided hemicranial region. The general examination was normal.

No fever, no weight loss, and no red flag symptoms. Neurological examination revealed a conscious, alert patient with normal cranial nerves and fundus, vision normal with no focal neurological deficits. MRI Brain Contrast was done to rule out secondary causes of headaches.

A rim of the abnormal subdural signal along the left cerebral convexity and left para-cerebellar region with smooth thick pachymeningeal enhancement consistent with pachymeningitis. CSF analysis revealed an aseptic picture with slightly raised protein.

All work up for vasculitis, tuberculosis, sarcoidosis, cryptococcosis, fungal, bacterial and viral malignant cytology was negative. The clinical and radiological picture was suggestive of Hypertrophic Pachymeningitis. IgG4 levels were raised, so the final diagnosis was IgG4 disease (IG4 associated hypertrophic pachymeningitis).

The patient was started on intravenous steroid pulse for three days with conservative treatment for headaches. The patient responded in 5 days and was discharged in a stable, headache-free state.

A rare disease, clinically hypertrophic pachymeningitis can present as debilitating headaches and cranial nerve defects but, in other cases, completely asymptomatic. Differentials included Tuberculosis, Sarcoidosis, Vasculitis, connective tissue disorder, fungal, bacterial, viral, malignant and cryptococcal. Several reports suggest a favourable response if treated for early IgG4 disease with steroids. It is a benign and treatable condition. The long-term treatment response and clinical course remain unknown.