Correlation of Variable Movement Disorders with Neuro-physiological Studies and Neuro-imaging Modalities in Paediatrics

Abstract

This study was conducted on 60 patients suffering from different types of involuntary movements. They were 25 patients presenting with ataxia (7 patients with FA, 13 patients with EOCA and 5 patients with static causes of ataxia; 2 patients with ataxic cerebral palsy, 2 patients with congenital cerebellar ataxia and one patients with post-encephalitic ataxia), and 35 patients presenting with different forms of extra-pyramidal involuntary movements; 12 patients with dystonia, 13 patients chorea and 10 patients with myoclonus. Some of these patients had progressive neuro- degenerative etiology e.g., white matter neuro-degenerative diseases, Wilson disease and mitochondrial diseases and others had static neuro-encephalopathic causes of the movement disorder, e.g. rheumatic cause of chorea, cerebral palsy, post-encephalitic or post­ traumatic. The study also included 10 age and sex matched controls.

Patients and controls were subjected to neurophysiological tools of investigations in the form of nerve conduction studies (motor and sensory), and somatosensory evoked potentials. Patients were also subjected to digital EEG and to neuro-imaging studies in the form of MRl to visualize the posterior fossa structures as well as the basal ganglia and white matter changes.

Regarding patients presenting with neuro-degenerative ataxias, patients with FA demonstrated a statistically significant slowing of motor and sensory conduction velocities,. delay of motor and sensory latencies and reduction of motor and sensory amplitudes in their nerve conduction studies compared to controls (P< 0.05), indicating both demyelinating and axonal patterns of neuropathy (motor and sensory). However, patients with EOCA demonstrated only a statistically