Isolated Neurosarcoidosis-A Diagnostic Enigma

Abstract

Neurosarcoidosis is a rare, but well-recognized cause of hypopituitarism with a predilection for the hypothalamus. We describe a case of panhypopituitarism in a 57-yr-old Asian lady, associated with an infiltrating hypothalamo-hypophyseal lesion, and other intracranial deposits, initially diagnosed as cerebral tuberculomata. Despite antituberculous therapy, the intracranial lesions progressed with significant clinical deterioration. Repeated lumbar puncture, magnetic resonance imaging scans, liver biopsy and Gallium scan were noncontributory, and the diagnosis of isolated neurosarcoidosis was established only following biopsy of an intracranial lesion. The lesion regressed on steroid and azathioprine therapy. Isolated neurosarcoidosis poses a considerable management problem. We review recent advances in the investigation, diagnosis, and treatment of this condition.

Hypothalamic–pituitary sarcoidosis with vision loss and hypopituitarism: case series and literature review

“…Neurosarcoidosis (NS) is defined by central or peripheral nervous system involvement. Although NS is found in 14-50 % of patients with systemic sarcoidosis on autopsy, it is clinically evident only in 5-13 % [2][3][4][5]. Clinical NS carries a worse prognosis with mortality of 10-18 % [6,7].…”

“…NS usually presents with cranial and peripheral nerve palsies, most commonly involving the facial nerve. Also, gray matter lesions can occur leading to seizures and psychiatric manifestations [4,[8][9][10]. Less commonly, the hypothalamic-pituitary (HP) region is involved, which may lead to pituitary hormone abnormalities (PHAs).…”

MRI differential diagnosis of suspected multiple sclerosis

“…Isolated neurosarcoidosis is very rare. 33 Neurosarcoidosis can affect any part of the CNS to clinically mimic a wide range of conditions.…”

Isolated spinal neurosarcoidosis: an autopsy case

“…[4][5][6] In addition, autopsy cases of such spinal isolated neurosarcoidosis were extremely rare or none.…”

“…[1][2][3] In addition, isolated spinal neurosarcoidosis sparing other organs is very rare. [4][5][6] Furthermore, autopsy cases of isolated spinal sarcoidosis are extremely rare or none. The author herein reports an autopsy case of isolated spinal neurosarcoidosis with an emphasis on pathological findings.…”