Isolated degeneration of the posterior column as a distinct entity--a clinical and electrophysiologic follow-up study.

OBJECTIVE

The aim of the study was to better describe the long term clinical course and electrophysiologic and radiologic findings in isolated degeneration of the posterior column.

METHODS

Four patients with the presenting symptoms of a progressive tabetic ataxia were followed up clinically and electrophysiologically over up to 15 years between 1997 and 2008. They received standardized neurological examinations, electrophysiologic testing with SEP, MEP, NCV, EMG, autonomic testing and cardiac evaluation, head and spine MRI, laboratory evaluation including CSF analysis.

RESULTS

Progressive gait ataxia due to pallhypasthesia and loss of position sense with areflexia remained the only symptoms. Pes cavus deformity was a notable clinical feature in all cases. There was no involvement of other systems and all patients remained fully ambulatory. There was no cardiac involvement. Electrophysiology was characterized by absent cortical tibial SEP with normal lumbar complexes and normal nerve conduction studies and transcortical magnetic stimulation as well as sympathetic skin response. MRI of the cord was normal. Laboratory analysis and CSF were unrevealing.

CONCLUSION

Isolated degeneration of the posterior column is a rare condition with a clinically benign course without progression involving other systems and characteristic electrophysiologic findings (isolated loss of cortical tibial-SEP with normal lumbar leads). Pes cavus deformity seems to be an unusual but typical clinical feature. The etiology is most likely a sporadic degenerative disease of the cord.