ABSTRACT:
We report a case of neurosarcoidosis in a patient presenting with rapidly progressive dementia and intermittent delirium. Sarcoidosis with neurologic involvement is rare, and typically does not manifest with psychiatric or cognitive symptomatology. The most common presentation of neurosarcoidosis is facial neuropathy, followed by meningoencephalitis. This patient exhibited accelerated mental status decline over several months. Her response to corticosteroid therapy was dramatic. This case report illustrates the importance of continual diagnostic vigilance in evaluation of dementia with an unusual clinical picture.
CASE:
A 61 year old Egyptian …show more content…
All systems should be thoroughly evaluated and invasive investigation should focus on the least invasive sampling location. Positive findings on chest imaging are present 25-65% of neurosarcoidosis …show more content…
Depending on the portion of the brain affected by granulomatosis, clinical presentation may range from apathy, to poor executive function, memory impairment or overt psychosis. Interestingly in our case, the patient had a combination of neurologic manifestations – dementia as well as aspects of delirium. The utility of MRI and CSF sampling is debatable, especially in patients who have extraneural involvement. Their use should be limited to cases where isolated CNS sarcoidosis is strongly suspected; and even then both tests lack sensitivity and specificity. Similarly, serum and CSF angiotensin converting enzyme levels should be interpreted with caution, as they can be affected by infection and/or malignancy.
The mainstay of therapy remains systemic corticosteroids, although there lacks quality evidence from placebo-controlled trials. The dose and duration of treatment is dictated by disease severity and response. Rapidly deteriorating patients may benefit from parenteral methylprednisolone followed by a slow tapering course of prednisone. Two thirds of patients follow a monophasic course, with progressive disease being less common. In certain cases if a definitive diagnosis cannot be obtained by tissue sampling, it may be necessary to commence treatment and follow the patient over