A Case and Literature Review of Normal Pressure Hydrocephalus in Mixed Connective Tissue Disease

Normal Pressure hydrocephalus (NPH) is characterized by gait apraxia, urinaryincontinence, and dementia. Mixed connective tissue disease (MCTD) is anautoimmune connective tissue disease that has never been reported to causeNPH. Our patient was a 67-year man with a one-year history of gradualworsening gait and balance, urinary urgency with urge incontinence anddecreased short-term memory. Previously he was diagnosed with mixedconnective tissue disease (MCTD). For him an endoscopic thirdventriculostomy (ETV) with brain biopsy was agreed upon. Gliotic brainparenchyma and focal perivascular lymphocytes were noted in the gray matter.There was no lymphocytic infiltration in brain parenchyma.Immunohistochemical stains demonstrated that the mononuclear inflammatoryinfiltrate was limited to several small cortical blood vessels surrounded by CD3T-cell lymphocytes with no vessel wall invasion or necrosis. No CD20 B-celllymphocytes were observed. Viral inclusions, microglial nodules, granulomas,and amyloid deposits in the blood vessels were not noted. In summary, therewas no definitive evidence of vasculitis. However, the presence of aperivascular lymphocytic infiltrate around the cortical vessels was unusual .Considering this probable co-occurrence suggests the need for a protocol tobetter evaluate idiopathic NPH in patients with an underlying autoimmunecomponent.

کلیدواژه ها:

Hydrocephalus ، Normal Pressure ، Mixed Connective Tissue Disease