Primary Lateral Sclerosis Plus Corticobasal Syndrome: a case series (P7.039)

OBJECTIVE: We describe the clinical features of patients meeting criteria for Primary Lateral Sclerosis (PLS) who later develop a Corticobasal Syndrome(PLS-CB). BACKGROUND: PLS is a rare pure upper motor neuron presentation of motor neuron disease. PLS patients’ life expectancy is longer than those with ALS with a reported mean survival of 16 years. CB is a progressive neurodegenerative disorder typically affecting one side (akinesia, extreme rigidity, dystonia, apraxia or alien limb phenomenon). Cases of PLS progressing into PLS-CB have yet to be described. METHODS: We searched our Neuromuscular Research Database for patients meeting clinical criteria for PLS between 1997 and 2010. We describe clinical features, initial PLS presentation, delay between PLS and CB symptom appearance, imaging, electrodiagnostic results and survival data. RESULTS: We identified 35 PLS patients, 3 of whom went on to develop PLS-CB (8.6[percnt]) 2 men, 1woman, ages 57-67 years. A memory or movement disorder specialist confirmed the diagnosis of CB, with symptoms developing 4-7 years after PLS diagnosis. EMG studies showed 1 patient with fibrillations in the left gastrocnemius stable over 5 years, and another patient with sensorimotor axonal neuropathy. Brain MRI scans showed asymmetric cortical atrophy in the right hemisphere in 2 patients, and atrophy of the left anterior temporal lobe in third patient. Of our PLS-CB patients, 1 patient died after 9 years of follow up, 5 years after diagnosis of CB, cause unknown. The other 2 patients have a total of 13 and 9 years of follow up, 6 and 5 years after CB diagnosis, respectfully. CONCLUSIONS: In addition to PLS with parkinsonian features or fronto-temporal dementia, we propose that PLS-CB is another association with this rare motor neuron disease. We speculate that the pathophysiology of this combination could be the same mechanism as those with ALS-FTD and PLS-FTD. Disclosure: Dr. Johnson has nothing to disclose. Dr. Dimachkie has received personal compensation for activities with Pfizer, Depomed, Merck, CSL Behring, NuFACTOR, BioMarin Pharmaceutical, Baxter International, Inc., and Catalyst Pharmaceuticals as a consultant, speaker, and/or advisory board member. Dr. Pasnoor has nothing to disclose. Dr. Statland has nothing to disclose. Dr. Jawdat has nothing to disclose. Dr. Barohn has received personal compensation from Grifols, Genzyme, and NuFactor. Dr. Barohn has received personal compensation in an editorial capacity for Neurology Clinics of North America. Dr. Barohn has received research support from Cytokenetics. Dr. Glenn has nothing to disclose. Dr. Dubinsky has received personal compensation for activities with Allergan, Inc. as a speaker. Dr. Burns has received research support from Janssen, Wyeth, Pfizer, Danone, Baxter, and Lilly/Avid Radiopharmaceuticals. Dr. McVey has nothing to disclose.