A 40 year-old right-handed man complained of dysfunction in his right arm of about 6 month’s duration. Onset was gradual and associated with slurring of speech, shaking of his left hand during use, and coughing and sputtering when swallowing liquids. His father and brother had similar symptoms: after 10 years, his father became confined to a wheelchair. The patient has never consumed alcohol.The general physical exam and mental status were normal. Speech was dysarthric. Coarse nystagmus was evoked by conjugate gaze in any direction. Smooth pursuit was abnormal and saccades were dysmetric. Sensory exam was normal. Tone was mildly diminished in all limbs. No resting tremor was noted, but there was a side-to-side intention tremor of both hands, right greater than left. There was minimal difficulty with the heel-to-shin maneuver. Rhythmic hand motions were decomposed (i.e. the patient exhibited dysdiadochokinesis). Muscle strength and muscle stretch reflexes were normal. Babinski’s sign was not present. Gait was somewhat broadbased and unsteady.
Localize the lesion or lesions and What is the likely diagnosis in this case?
Det var en svær en, for mig i det mindste.
hvad synes i?