Isolated frontal disequilibrium as presenting form of anti-Hu paraneoplastic encephalomyelitis.
Anti-Hu encephalomyelitis is one of the most frequent paraneoplastic syndromes, classically presenting with diffuse neurological involvement. We report a 69-year-old man presenting with a three-month isolated, progressive gait disorder with normal neurological examination, except for loss of balance and gait failure reminding frontal disequilibrium, only accompanied by a very mild rigidity of his right foot. MRI of the brain showed hyperintensities in both amygdale and left putamen. EMG study showed no abnormal continuous spontaneous fiber activity. Because of fast progression and MRI findings, anti-Hu antibodies were tested, resulting positive. Mediastinal biopsy of two adenopathies detected by body-PET, confirmed an oat-cell carcinoma. The patient received oral steroids and oncological therapy. One year later, the tumor is in remission. His gait and abnormal posture of right leg are normal. Only mild residual hyperintensities persist on follow-up MRI. A paraneoplastic syndrome should be considered in the differential diagnosis of subacute, fast progressive gait disorders.
Compta
Yaroslau
Y
Department of Neurology, Institute of Neurosciences, Hospital Clínic i Universitari de Barcelona, Barcelona, Catalonia, Spain.
Valldeoriola
Francesc
F
Urra
Xabier
X
Gómez-Ansón
Beatriz
B
Rami
Lorena
L
Tolosa
Eduardo
E
Graus
Francesc
F
D000368:Aged; D000679:Amygdala / Q000473:pathology; D001323:Autoantibodies / Q000097:blood*; D018288:Carcinoma, Small Cell / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology / Q000628:therapy; D003131:Combined Modality Therapy; D051959:ELAV Proteins / Q000276:immunology*; D000067779:ELAV-Like Protein 4; D005500:Follow-Up Studies; D020235:Gait Apraxia / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D006801:Humans; D008175:Lung Neoplasms / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology / Q000628:therapy; D008198:Lymph Nodes / Q000473:pathology; D008207:Lymphatic Metastasis / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D020361:Paraneoplastic Syndromes, Nervous System / Q000175:diagnosis / Q000276:immunology / Q000473:pathology; D011699:Putamen / Q000473:pathology
D001323:Autoantibodies; D051959:ELAV Proteins; D000067779:ELAV-Like Protein 4; C498010:ELAVL4 protein, human