Abstract Details

Title Stroke-like Presentation with Acute Spastic Quadriparesis Revealing Adult Polyglucosan Body Disease (APBD)

Topic General Neurology

Presentation(s) P5 - Poster Session 5 (8:00 AM-9:00 AM)

Poster/Presentation
Number 6-004

Objective NA

Background Adult polyglucosan body disease (APBD) is a rare metabolic disorder that typically presents between the fourth and seventh decades, and is characterized by peripheral neuropathy, progressive motor weakness/spasticity with gait disturbance, neurogenic bladder, and cognitive impairment. The diagnosis is suggested by typical imaging findings and confirmed with genetic testing and pathologic biopsy. We report a novel presentation of this disease in a 65-year-old man with acute neurological symptoms mimicking stroke, in the absence of cardiovascular risk factors.

Design/Methods NA

Results A 65-year-old man with progressive gait disturbance attributed to obesity presented with acute unresponsiveness and quadriplegia. On arrival the patient was acutely hypertensive with no movement in bilateral extremities and a right gaze preference. Admission stroke scale was 25 with initial concern for acute basilar artery stroke. Neuroimaging did not show acute infarct or significant vascular stenosis or occlusion. Computed tomography of the head showed small right fronto-parietal subdural bleed resulting from trauma. Magnetic resonance imaging of the brain and cervical spine showed a pattern consistent with a leukodystrophy syndrome. The patient’s clinical exam failed to improve after two weeks, and he was eventually extubated to comfort measures. Genetic testing and postmortem pathologic examination confirmed a diagnosis of APBD.

Conclusions APBD is typically recognized as a progressive neurologic disorder with characteristic symptomology, and rarely presents with rapid deterioration in an acute presentation as the case described here. The patient likely had a more gradual progression of neurologic symptoms that had not previously been recognized. His course progressed rapidly in setting of acute trauma. APBD was suspected after routine stroke workup suggested a leukodystrophy syndrome, and confirmatory testing supported the diagnosis. This case suggests APBD and similar conditions should be considered in cases of rapidly progressing neurologic deterioration encompassing a broad constellation of symptoms.

Authors/Disclosures
Jaspreet Johal, MD PRESENTER	Dr. Johal has nothing to disclose.
Ramiro G. Castro Apolo, MD (Lehigh Valley Health Network)	Dr. Castro Apolo has nothing to disclose.
Nathan P. Fenstermacher (Lehigh Valley Health Network)	Mr. Fenstermacher has nothing to disclose.
	No disclosure on file
Adam B. Edwards, MD	Dr. Edwards has nothing to disclose.
Preet M. Varade, MD (Lehigh Valley Hospital - Cedar Crest)	Dr. Varade has nothing to disclose.

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