Case 2 history, examination
Female 61 years old patient was admitted to our neurology department in 2019. Patient’s complains consisted progressive muscle spasticity and weakness more prominent in lower limbs, muscle atrophy, speaking and swallowing disturbances. First symptoms developed in 2012 when patient noticed walking difficulty due to spasticity and weakness in the right leg. In 3 months muscle weakness involved the left leg. The disease progressed slowly, and patient had to use a walking stick in 2014, and then a wheelchair in 2016. In 2014 hypotrophy of the interosseous muscles of the hands, and thenar hypotrophy were noted. Within the period 2012 to 2018 the disease progression was slow and included only worsening low limbs muscle weakness and spasticity. During 2014-2016 the patient had urge urinary incontinence. In 2018 muscle weakness involved the right hand, in 2019 muscle weakness involved the left hand and bulbar muscles (dysphonia, dysphagia).
In 2019 neurological examination revealed tetraparesis with severe muscle weakness in lower limbs up to 2/5 points and mild weakness in upper limbs up to 4/5 points. The patient was not able to stand or walk by herself. Tendon reflexes were brisk with wide spread. Rare fasciculations in limbs, pronounced tongue fasciculations, moderate muscle atrophy of the all limbs, speaking and swallowing disturbances, pathological signs, and spasticity more prominent in lower limbs were noted. There were no sensation disturbances.
Across the disease course the patient underwent MRI several times, EMG, and TMS. In 2012-2013 MRI of the lumbar spine revealed the spinal cord without any pathology. MRI of the cervic and thoracic spine revealed dilated central canal at the C6-Th1 vertebrae level and slightly narrow of vertebral canal (vertebral canal is less than vertebrae’s front-rear size) in 2014. The spinal cord anterior horns damage signs in form of both motor unit remodeling and denervation were revealed by EMG in 2014 and 2015. Also in 2015 TMS showed motor pathway dysfunction at the cortical level. In 2019 dilated central canal of the cervical spinal cord remained in the same size (C6-Th1) by MRI (Fig.5). Laboratory tests of blood and CSF were normal.