Mills' Syndrome - A Clinical Variant -Case Report

The Mills' syndrome was first described by Mills in 1900, the syndrome included progressive, ascending or descending hemiplegia, with no significant sensory impairment. Opposing to be a variant of primary lateral sclerosis, this syndrome is accepted as degeneration of the corticospinal tract unilaterally. Fifty six years old, right handed female patient was admitted to our clinic outpatient outpatient clinic with left spastic hemiparesis, dysphagia, dysarthria and bilaterally prominent lower extremity spasticity and behavioral disturbances, sudden loughing, spastic contractility of arms and legs, gait disturbance for the last three years. Following the dysartria, the loss of power belonging to left lower extremity was proceeded by spasticity prominently left side and cognitive impairment with slow progression. On neurological evaluation, she was concious and cooperative partially. The cranial nerves examination were yielded dysphagia and dysphonia, there were spastic plegia on upper and lower left sided extremities. Deep tendon reflexes were hyperactive with Babinski sign on the right. The cerebral MRI showed mild generalised atrophy prominently right sided, cervical MRI showed both spondylosis and significant spinal cord atrophy. EMG investigation revealed significant neurogenic motor unit changes on left sided extremities especially lower area. Our case is carrying a rare clinical specifications of hemiplegic ascending ALS type of Mills and also unilateral cerebral atrophy is discussed in the light of the literature.