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Anti-Ma2 associated paraneoplastic neurological syndrome presenting as encephalitis and progressive muscular atrophy

¹ Division of Neurology, JR Tokyo General Hospital, 2-1-3, Yoyogi, Shibuya-ku, Tokyo 151-8528, Japan
² Department of Neurology, Kyorin University School of Medicine, Mitaka-shi, Tokyo 181-8611, Japan
³ Department of Neurology, Tokyo Metropolitan Matsuzawa Hospital, Tokyo 156-0057, Japan
⁴ Department of Neurology, Brain Research Institute, Niigata University, Niigata 951-8585, Japan

Correspondence to:
Correspondence to:
Dr M Waragai
Division of Neurology, JR Tokyo General Hospital, 2-1-3, Yoyogi, Shibuya-ku, Tokyo 151-8528, Japan; warawaram2002{at}aol.com

ABSTRACT
A 36 year old man with a history of testicular germ cell tumour presented six months after bilateral orchidectomy with progressive amnesia, irritability, vertical gaze palsy, and generalised seizures. Eight months after initial onset of symptoms, he demonstrated a head drop with muscular atrophy of the upper limbs, shoulder girdle, and posterior neck. He reported no sensory disturbances and his sensory examination was normal. The overall clinical presentation was consistent with motor neurone disease. Cerebrospinal fluid analysis revealed mild pleocytosis and increased protein concentration. Serum and cerebrospinal fluid were positive for the anti-Ma2 antibody by western blot analysis and immunostaining. Abnormal high signal in the grey matter was noted in the cervical spinal cord and brain by T2 weighted magnetic resonance imaging (MRI). The patient was treated with corticosteroids, intravenous immunoglobulin, and antiepileptic medication. The patient improved clinically and symptom progression ceased after initiation of treatment. There was complete resolution of the abnormal brain MRI lesions; however, the cervical spinal cord MRI lesion and muscular atrophy remained unchanged. It is suggested that the anti-Ma2 antibody is involved not only in encephalitis, but may also play a role in the cervical spinal cord lesions resulting in a motor neurone disease-like presentation.

Abbreviations: CSF, cerebrospinal fluid; MRI, magnetic resonance imaging

Keywords: paraneoplastic neurological syndrome; anti-Ma2 antibody; encephalitis; muscular atrophy; spinal cord; magnetic resonance imaging

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