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Figure 1: Brain and spine image and electromyography of the ALS case. (A) Brain MRI suggests Arnold-Chiari malformation type I (arrow). Spine
MRI reveals slight prominence of intervertebral disks at C4/5, C6/7 and L4/5-L5/S1, and no abnormality was observed in the whole spinal cord. (B-D)
Electromyography showed denervation potentials in the left biceps brachii (B), the left thenar muscle (C) and right gastrocnemius (D). ALS: amyotrophic
lateral sclerosis; MRI: magnetic resonance imaging
progressed to her right lower limb and left upper limb both sides. For laboratory examinations, blood cell
in turn. Muscle cramps could be induced by touching count, urine analyses, liver function, renal function,
her skin of limbs and chest. Muscle cramps frequently muscle enzymes (creatine kinase, creatine kinase-MB,
broke out during sleeping, and could be alleviated aspartate aminotransferase, lactate dehydrogenase,
slightly by standing. Stiffness and weakness developed hydroxybutyrate dehydrogenase), serum vitamin B12,
in the lower limbs for 4 months after symptom onset. autoantibodies and tumor biomarkers were normal.
Fasciculations, combined with tight feeling of the hips, Intracranial pressure and cerebral spinal fluid analysis
was seen in both lower extremities and left upper limb. were normal. Brain and spine magnetic resonance
Then she had difficulty in walking, button-up her imaging (MRI) suggest Arnold-Chiari malformation
clothes and bradykinesia. There was no numbness and type I [Figure 1A], which was not explainable for the
muscle atrophy of her tongue, face, hands and limbs. symptoms and abnormalities of physical examinations.
She had no complaints of dysphagia, dysarthria or Spine MRI revealed slight prominence of intervertebral
dyspnea and history of alcohol drinking or cigarette disks and no abnormality was observed in the whole
smoking, no family history of neurological diseases. spinal cord [Figure 1A]. Electromyography (EMG)
showed neurogenic changes in the left biceps brachii,
Physical examination revealed a normal body the left thenar muscles and bilateral gastrocnemius
figure, well-oriented in place, time and person, with [Figure 1B-D]. Evoked potentials examination showed
no unconsciousness. Cranial nerve examination prolonged latency period in the left side of the visual
revealed no abnormalities. Examination of limbs evoked potential and event-related potential-P300.
revealed spastic rigidity in both lower limbs, Motor evoked potential, somatosensoroy evoked
with 4/5 muscle power in both lower limbs, 4/5 potential and brainstem auditory evoked potential,
muscle power in left upper limb, and 4/5 to 5/5 nerve conduction, ambulatory electroencephalogram
muscle power in right upper limb. There was no and electrocardiogram were normal.
atrophy of the limbs, trunk and lingual muscles.
Sensations and cerebellar examinations were According to the revised 2,000 criteria, the patient
[12]
unremarkable. Deep tendon reflexes in the 4 limbs was diagnosed with clinically probable ALS, which
were asymmetrical that left limbs were hyperactive was confirmed by other hospitals in Beijing. The
and right limbs brisk. Hoffman’s reflexes and oral administration of diazepam and baclofen was
patellar clonus were negative. Left ankle clonus initiated; muscle cramps were relieved a bit. The
was positive. Babinski’s signs were presented in patient did periodic follow-ups in our outpatient
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