A 51-year-old man, with a 20-year history of heavy alcohol abuse (1.5 L of beer per day for 20 years often accompanied by 360 mL of shochu, a Japanese distilled spirit containing 25-35% alcohol by volume) and loss of appetite for 4 years, progressively developed slurred speech for 3 weeks. Subsequently, he choked while drinking and had difficulty swallowing food. Finally, he could not eat or drink, and was admitted to our department. He had previously been diagnosed with alcoholism and had a history of chronic obstructive pulmonary disease. At the age of 44, he underwent burr-hole drainage for bilateral chronic subdural hematomas. After surgery, he became independent regarding the activities of daily living. There was no family history of MND. On admission, blood pressure was 112/79 mmHg, and body height and weight were 183 cm and 48 kg, respectively. Neurological examination revealed an alert patient with a mini-mental status examination (MMSE) score of 22 points (orientation to time, -2 points; attention and calculation, -4; three word recall, -2). There was horizontal gaze paretic nystagmus bilaterally. The facial expression was flat, and there was weakness of the orbicularis oris bilaterally. Weakness of the frontalis muscle and orbicularis oculi was not found. The speech was slurred, and there was difficulty swallowing; the gag reflex and palate elevation were poor. The jaw jerk reflex was brisk and the snout reflex was positive. Emotional lability was not found. Neither tongue atrophy nor fasciculation were found. The tongue could be protruded from the mouth and remained midline, and moved adequately from side to side. Motor weakness was evident, with scores of 4 in the distal upper limbs bilaterally and 3 in the proximal lower limbs bilaterally on the Modified Medical Research Council’s manual muscle test (MMT). There was finger-to-nose incoordination bilaterally. The patient could not walk because of a wide-based gait with truncal instability. Deep tendon reflexes in the upper limbs were increased, and Babinski reflexes were positive bilaterally. Hoffmann reflexes and the forced grasp reflex were negative. The superficial vibratory and position senses were normal.
The initial diagnosis was MND because of the development of progressive dysarthria and dysphagia with upper and lower motor neuron signs, and the limb weakness with upper motor neuron involvement. However, a nerve conduction study did not reveal prolonged distal latencies, conduction blocks, or an absent F-wave. Needle electromyography showed that normal unit potentials and no denervation potentials were found in the tongue, sternocleinomastoid, biceps or quadriceps femoris. Brain MRI demonstrated hyperintense lesions in the precentral gyrus bilaterally and in the splenium of the corpus callosum on FLAIR (Figure A). Those lesions were also hyperintense on DWI (Figure B), and the apparent diffusion coefficient (ADC) map did not show significant signal changes (Figure C). Laboratory test results included decreased vitamin B1 (14 ng/mL; normal: >24 ng/mL), and mild elevation of liver enzymes. The cerebrospinal fluid was normal.
Figure 1 Admission MRI. Representative images of brain MRI on admission including fluid attenuated inversion recovery (FLAIR) (A), diffusion-weighted image (DWI) (B), and the apparent diffusion coefficient (ADC) map (C), showing hyperintense lesions in the precentral (more ...)
Complex vitamin B therapy, including 100 mg of thiamin, was started intravenously on the day of admission. After admission, the patient’s swallowing slowly improved, and gradually the speech became clear. On admission, only food of a pudding-like texture was tolerated, but 7 days after admission gruel-like foods were manageable, and 13 days after admission the patient was placed on a normal diet. On hospital day 14, the MMSE score had increased to 26 points (attention and calculation, -4), limb weakness had improved, and the patient could walk with a cane. Concurrently, hyperreflexia of the jaw jerk and bilateral upper limb reflexes were normalized, and the bilateral Babinski reflexes became negative. Gaze paretic nystagmus and finger-to-nose incoordination were also improved. Repeat MRI at 17 days after admission showed the disappearance of signal abnormalities in the splenium of the corpus callosum and the precentral gyrus on FLAIR and DWI (Figure A-C).
MRI after therapy. Representative FLAIR images (A), DWI (B), and ADC map (C) 17 days after admission, showing disappearance of the hyperintense lesions.