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Figure 6: A 24 year old male presented with acute onset headache with drowsiness in the background of recent fever. On examination he had pupil sparing ptosis,vertical gaze paresis, mild sensory neural deafness and bilateral subtle pyramidal signs. MRI brain showed symmetric hyperintense lesions in the caudate nuclei and putamina extending into the thalami and periaqueductal regions, on T2W images (Figure 6). His serum and CSF lactic acid levels were raised.A quadriceps mucle biopsy revealed subsarcoplasmic accumuilation of mitochondria) associated with cytochrome oxidase negative fi bres. No mitochondrial mutations were detected. Following intravenous Thiamine therapy he recovered rapidly

Figure 6: A 24 year old male presented with acute onset headache with drowsiness in the background of recent fever. On examination he had pupil sparing ptosis,vertical gaze paresis, mild sensory neural deafness
and bilateral subtle pyramidal signs. MRI brain showed symmetric hyperintense lesions in the caudate nuclei and putamina extending into the thalami and periaqueductal regions, on T2W images (Figure 6). His serum and CSF lactic acid levels were raised.A quadriceps mucle biopsy revealed subsarcoplasmic accumuilation of mitochondria) associated with cytochrome oxidase negative fi bres. No mitochondrial mutations were detected. Following intravenous Thiamine therapy he recovered rapidly