The clinical course of progressive bulbar palsy

Abstract

Our objective was to study the clinical course of patients diagnosed with progressive bulbar palsy (PBP). We reviewed all 392 medical records of ALS patients seen between 1 January 2000 and 31 July 2007. Patients with isolated PBP at presentation were selected and classified into those with normal EMG of the limbs (PBP-N) and those with active denervation on EMG (PBP-A). We studied the time to progression of these patients to ALS. We compared patients with PBP-N to patients with PBP-A. Fifteen patients were diagnosed with PBP-N. The remaining 17 had PBP-A. Thirteen of the 15 patients with PBP-N (87%) progressed to definite ALS. The two patients who did not progress to ALS died at 22 and 60 months, respectively. The median survival time was 35 months for the PBP-N group and 40 months for the PBP-A group (p = 0.92). Except for the rate of depression, patients with PBP-N did not differ from patients with PBP-A in the basic demographics, time of presentation, clinical course, survival and treatment received. All patients with FTD died within 40 months of onset of symptoms. In conclusion, almost all PBP patients progress to ALS regardless of the presence of upper motor signs or generalized denervation on EMG of the limbs.

Key words::

• Amyotrophic lateral sclerosis
• progressive bulbar palsy
• frontotemporal dementia

Acknowledgements

We thank Patricia Friedmann for the statistical analysis. We also thank Weiyi Gao, Andres Deik, Aamir Hussain, Biggya Sapkota, Theresa Imperato, and Joel Bernor who contributed to the data collection. We thank Frida Chehlaoui and Jill K. Gregory for assistance with the graphics.

Declaration of interest: Dr. Karam serves on the editorial team for the Neurology(r) Resident and Fellow Section. Drs. Scelsa and MacGowan have nothing to disclose. The authors have no conflict of interest.