Unilateral positive-negative myoclonus in Creutzfeldt-Jakob disease

• Published in: Movement disorders Vol. 21; no. 11; pp. 1963 - 1966
• Main Authors: Ohnari, Keiko, Matsunaga, Kaoru, Uozumi, Takenori, Tamagawa, Akira, Hashimoto, Tomoko, Tsuji, Sadatoshi
• Format: Journal Article
• Language: English
• Published: Hoboken Wiley Subscription Services, Inc., A Wiley Company 01.11.2006; Wiley
• Subjects: Biological and medical sciences; Brain - pathology; Brain - physiopathology; Creutzfeldt-Jakob disease; Creutzfeldt-Jakob Syndrome - complications; Creutzfeldt-Jakob Syndrome - pathology; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Electroencephalography - methods; Electromyography - methods; Evoked Potentials, Motor - physiology; Functional Laterality; Headache. Facial pains. Syncopes. Epilepsia. Intracranial hypertension. Brain oedema. Cerebral palsy; Humans; Magnetic Resonance Imaging - methods; Male; Medical sciences; Middle Aged; Myoclonus - classification; Myoclonus - etiology; Myoclonus - pathology; Nervous system (semeiology, syndromes); Neurology; positive-negative myoclonus; positive-negative myoclonus; Prion disease; Nervous system diseases; Myoclonus; Creutzfeldt- Jakob disease; Cerebral disorder; Involuntary movement; Infection; Central nervous system disease; Degenerative disease; Prion; Neurological disorder; Creutzfeldt Jakob disease
• ISSN: 0885-3185; 1531-8257
• DOI: 10.1002/mds.21078

We report a patient with Creutzfeldt–Jakob disease who presented asymmetric myoclonus. Positive–negative myoclonus was seen only in the right extremities in association with periodic synchronous discharges (PSDs) on the electroencephalogram, although pure positive myoclonus was rarely seen in the left extremities, independently in PSDs. The duration of the silent period recorded in the right‐hand muscle produced by transcranial magnetic stimulation was much longer than that in the left‐hand muscle or that in normal subjects. Brain MRI diffusion‐weighted images showed signal hyperintensities in the putamen and cerebral cortex, including the motor cortex, predominantly on the left side. © 2006 Movement Disorder Society