Correspondence Address:
Dr. Abhishek Juneja
Department of Neurology, Dr. RML Hospital, New Delhi - 110 001
India

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Dear Editor,

We report a case of 46-year-old female patient with headache for 10 days, left facial drooping for 4 days and double vision for 2 days. She had a history of similar headache with difficulty swallowing and hoarseness of voice lasting for a few weeks 6 months ago, which improved following treatment. There was no history of any comorbid illness including diabetes. On neurological examination, she had left-sided lateral rectus and peripheral facial nerve palsy. Rest of the neurological examination was normal. Routine blood investigations including blood counts, renal, hepatic, thyroid, glycaemic, and autoimmune profile were normal; inflammatory markers such as erythrocyte sedimentation rate and C-reactive protein were high. HIV testing by enzyme-linked immunosorbent assay was negative. Chest radiograph and ultrasound abdomen were normal. Magnetic resonance imaging (MRI) of brain and cerebrospinal fluid (CSF) examination were unremarkable. Serum angiotensin converting enzyme (ACE) done in view of repeated episodes of multiple cranial neuropathy in a middle-aged nondiabetic female individual was high (116 U/L; N : 8-52 U/L). The patient was treated with intravenous methylprednisolone 1 g/day for 5 days followed by oral prednisolone at a dose of 1 mg/kg/day, keeping the diagnosis of neurosarcoidosis. Her headache, double vision resolved and she completely regained facial strength over the next 4 weeks.

Sarcoidosis is a multi-system granulomatous disease, most commonly affecting lungs. Neurosarcoidosis accounts for only 5%–15% of cases; generally, presenting with cranial mononeuropathy, with facial nerve being the most commonly involved followed by optic neuropathy.[1],[2] Other presentations include headache, seizures, raised intracranial tension, hypertrophic meningitis, myelitis, and peripheral neuropathy.[1],[2] Multiple cranial neuropathy is a rare presentation for neurosarcoidosis.[3] We ruled out other common causes of recurrent multiple cranial neuropathy namely multiple sclerosis, immune mediated or infective vasculitis, structural lesions including tumor by relevant investigations such as neuroimaging, CSF examination, and autoimmune profile.

The diagnosis of neurosarcoidosis relies on clinicoradiological correlation and histopathological confirmation of granulomatous inflammatory changes. MRI brain changes may include dural mass lesions, meningeal thickening, or post contrast leptomeningeal enhancement.[2] A normal MRI brain like in our case does not rule out the possibility of neurosarcoidosis. Extraneural tissue biopsy from a peripheral or hilar lymph node if obtained can establish the diagnosis in doubtful cases. Neural tissue biopsy is difficult to obtain and involves far more risk than an extraneural tissue biopsy. Serum ACE level, although not very sensitive but fairly specific marker of sarcoidosis can aid in supporting the diagnosis in cases where other confirmatory tests are absent or difficult to obtain, like in our case.[4] Treatment strategies mainly rely on the use of corticosteroid and immunosuppressive therapy; surgical intervention may be required in selected cases. We therefore suggest neurosarcoidosis should be considered as a differential diagnosis in a case of multiple cranial neuropathy to prevent fatal complications.

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