Journal Article

A case of flail limb amyotrophic lateral sclerosis with significant sensory loss on neurophysiological studies

Benjamin, Sacks, Maria Francisca, Rocha, Michelle, Balaratnam

2023

Overview

IntroductionThis case describes a 42-year-old man where diagnosis of flail limb variant of amyotrophic lateral sclerosis (ALS) was complicated by poorly controlled type 2 diabetes mellitus.Clinical PresentationA 42-year-old man with type 2 diabetes mellitus presented with a progressive, ascending weakness in his right upper limb. Within 6 weeks he had a complete monoparesis. Nerve conduction studies demonstrated demyelinating features and secondary axonal loss of all four limbs. Diabetic neuropathy was considered. MRI of the brachial plexus was unremarkable. After 5 months the patient presented to UCLH A&E with shortness of breath on exertion, dysphagia and dysarthria. When examined in Neurology SDEC, there was a lower motor neuron monoparesis affecting the right upper limb with tongue fasciculations. Hba1c was 106 mmol/mol (reference range 20-41 mmol/mol). Repeat neurophysiology fulfilled the Awaji lower motor neuron criteria of probable ALS1 consistent with a flail arm variant. There was an additional length dependent, sensory diabetic polyneuropathy.DiscussionCo-existent poorly controlled diabetes complicated the diagnostic pathway. This case high- lights the need for timely neurology assessment and discussion with neurophysiology when there is diag- nostic uncertainty. Clinicians should be aware of the potential impact of comorbidities on neurophysi- ological studies.Reference De Carvalho M, Dengler R, Eisen A, et al. Electrodiagnostic criteria for diagnosis of ALS. Clin Neurophysiol. 2008;119(3):497-50318164242

• Publisher: BMJ Publishing Group Ltd,BMJ Publishing Group LTD
• Subject: Amyotrophic lateral sclerosis / Association of British Neurologists: Annual Meeting Abstracts 2023 / Diabetes / Diabetic neuropathy / Neurophysiology