323 Is hypothalamic involvement truly a red flag for multiple sclerosis? Chandra Mohan Sharma MD DM, Alok Jain MD, DM , BL Kumawat MD DM, Dinesh Khandelwal MD DM, Deepak Jain MD DM Department of Neurology, Sawai Man Singh Medical College and Attached Hospitals, Jaipur, Rajasthan, India Abstract Any hypothalamic disturbance manifesting clinically is considered a major red flag for multiple sclerosis, whereas MRI lesions involving deep grey matter structures are considered an intermediate red flag. However, hypothalamic lesions manifesting clinically with hypersomnia have been described in some patients of multiple sclerosis. We report a case where the first and presenting feature of multiple sclerosis was acute onset hypersomnia with bilateral hypothalamic lesions. On review of recent literature, we also question whether clinical or radiological hypothalamic involvement is really so unusual that it should be considered a red flag for multiple sclerosis. Neurology Asia 2013; 18(3) : 323 – 325 Address correspondence to: Dr. Alok Jain, Department of Neurology, Sawai Man Singh Medical College and Attached Hospitals,Jaipur, Rajasthan, 302004 India. Tel no: +91 9887808055, Email: doc_alok@yahoo.co.in INTRODUCTION Classically, the white matter pathology is said to be predominant in multiple sclerosis (MS), at least in the early stages. In fact, MRI T2 hyper-intensities of the deep grey matter structures including basal ganglia, thalamus and hypothalamus are considered an intermediate red flag for the diagnosis of MS, and any hypothalamic disturbance manifesting clinically is considered a major red flag, i.e. pointing fairly definitively to a non-MS diagnosis. 1 Hypothalamic lesions may be the basis of autonomic or endocrine disturbances, as well as disturbances of arousal and sleep, which indeed are rare in MS patients. We describe a case where the first presentation of MS was with acute onset hypersomnia and bilateral hypothalamic lesions. We also review the literature for similar clinical presentations, and for radiological or histopathological evidence of hypothalamic involvement in MS. CASE REPORT A 16 year old old girl developed excessive daytime sleepiness over 2 to 3 days, 15 months ago, with spontaneous partial improvement in symptoms over next few weeks. Three months later, she developed unsteadiness of gait and slurring of speech. The MRI brain study done at that time showed T2 hyper-intensities in bilateral hypothalamic regions, lower brainstem, as well as two periventricular lesions along lateral ventricles. The patient was seen by a General Physician who administered an intravenous steroid pulse, keeping a working diagnosis of demyelinating disease, following which there was considerable improvement in symptoms. She first presented to our centre 8 months after the first event, with acute onset of head nodding movements along with tremulousness of limbs. On examination, bilateral optic disc pallor was noted. She also had cerebellar dysarthria, titubation, limb and gait ataxia. The MRI brain repeated at our centre revealed multiple new supratentorial lesions in juxtacortical and periventricular white matter and pericallosal regions, as well as infratentorial lesions involving bilateral middle cerebellar peduncles. MRI study of spinal cord was normal. CSF analysis showed raised proteins (99mg/dl) with normal glucose and cytology, absent oligoclonal bands and normal levels of angiotensin converting enzyme. The P 100 latencies of visual evoked potential both sides were prolonged. Routine blood investigations and the endocrine profile were normal. Serum aquaporin-4 antibody was undetectable. Further evaluation by a battery of tests for neurosarcoidosis, autoimmune disorders, vasculitis, neurosyphilis, brucellosis and AIDS was also normal. The patient was treated with intravenous methylprednisolone pulse (1 gram daily for five days), following which there was marked improvement in cerebellar and sleep disturbances. The patient was diagnosed as relapsing remitting MS (RRMS) and counselled