Abstract Details

Title Hemiplegic Form of Amyotrophic Lateral Sclerosis (ALS) with Cognitive Changes: An Autopsy Study

Topic Anterior Horn

Presentation(s) P02 - (-)

Poster/Presentation
Number 176

Objective

Background BACKGROUND: It is uncertain if the hemiplegic form of ALS (Mills Syndrome) is a variant of ALS, PLS, or FTD/ALS. Some consider it a form of corticobasal degeneration. Here we describe a patient with hemiplegic ALS with autopsy findings.

Design/Methods DESIGN/METHODS: Case report with autopsy findings.

Results RESULTS: A 67 year old man, an attorney, had nine months of slurred speech, progressive difficulty swallowing and shortness of breath. Meaningful speech subsequently ceased and percutaneous endoscopic gastrostomy was needed as was non invasive ventilation. Muscle twitching, poor handwriting, falls, episodes of uncontrolled laughing, behavioral disinhibition and agitation appeared. Sensation was normal and no family member had neurodegenerative disease. Exam showed left-sided spasticity with brisk reflexes, positive Babinski and Hoffmann signs but normal strength. There was limited upgaze, left central facial weakness, overactive gag and a jaw jerk, compatible with upper motor neuron predominant bulbar ALS, pseudobulbar affect and neurobehavioral involvement. Brain imaging showed non-specific changes. Blood studies were normal or non-specific. Neck CT, chest CT and EMG/NCS were normal. He died 33 months after onset of symptoms due to respiratory failure. Autopsy showed asymmetric corticospinal degeneration and replacement by macrophages, reactive gliosis, and ubiquitin positive intranuclear inclusions in the cortex, brainstem, and lateral columns of the spinal cord. There were no focal abnormalities in the frontal or temporal lobes.

Conclusions CONCLUSIONS: Our results suggest that despite the atypical clinical features of Hemiplegic ALS, pathology suggests that the findings are typical for what is found in patients with classical ALS.

Authors/Disclosures
Ayushi Chugh, MD, FAAN PRESENTER	Dr. Chugh has nothing to disclose.
Michael D. Frost, MD (Minnesota Epilepsy Group, PA)	No disclosure on file
Mona Shahbazi (Hospital for Special Surgery)	No disclosure on file
Dale J. Lange, MD, FAAN (Lange Neurology, PC)	No disclosure on file

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