Abstract
IntroductionWe report a rare case of fulminant vasculitic mononeuropathy resulting in brachial diplegia, with suspected brainstem and autonomic nervous system involvement in a patient with diabetes mellitus.Case presentationA 58-year-old Hispanic Caucasian man with diabetes mellitus presented with a 1-year history of progressive bilateral upper extremity weakness, orthostatic intolerance and progressive memory decline. Diagnostic evaluation including laboratory tests for progressive encephalopathies, systemic inflammatory and non-inflammatory neuropathies, cerebrospinal fluid analyses, electrodiagnostic studies, and nerve biopsy were performed. Clinical examination revealed moderate cognitive deficits on the Montreal Cognitive Assessment scale, bilateral facial weakness and weakness of bilateral shoulder girdle and intrinsic hand muscles. Cerebrospinal fluid analyses revealed elevated protein and an elevated immunoglobulin G synthesis rate, suggesting an immune-mediated process. Further laboratory work up was non-diagnostic. Electrodiagnostic studies demonstrated chronic asymmetric axonal mononeuropathies with ongoing denervation. A superficial radial nerve biopsy showed a chronic vasculitic neuropathy. Glucocorticosteroid treatment, symptomatic pharmacologic and supportive non-pharmacologic therapies resulted in improved clinical outcomes despite challenges with glycemic control.ConclusionsThis case report emphasizes the importance of a thorough evaluation of atypical or uncommon neuromuscular presentations in diabetic patients without etiological presumptions. This is necessary in order to promptly establish a diagnosis, initiate appropriate therapies and prevent irreversible nerve injury.
Highlights
We report a rare case of fulminant vasculitic mononeuropathy resulting in brachial diplegia, with suspected brainstem and autonomic nervous system involvement in a patient with diabetes mellitus.Case presentation: A 58-year-old Hispanic Caucasian man with diabetes mellitus presented with a 1-year history of progressive bilateral upper extremity weakness, orthostatic intolerance and progressive memory decline
We report the case of a patient who presented with severe progressive, asymmetric bilateral upper extremity weakness, associated with rapid onset cognitive decline, cranial mononeuropathies and orthostatic hypotension, in the setting of suboptimal diabetes mellitus control
Our patient presented with progressive mononeuropathy multiplex, dysautonomia and cognitive decline, with a known history of diabetes mellitus that was suboptimally controlled
Summary
We report an uncommon case of brachial diplegia, multiple cranial neuropathies, orthostatic hypotension and moderate cognitive impairment with electrophysiological evidence for chronic axonal mononeuropathy multiplex secondary to small vessel vasculitis in a diabetic patient. Mononeuropathy multiplex and vasculitis should be considered in diabetic patients irrespective of glycemic control status if these patients present with non-length dependent, asymmetrical motor or sensory deficits and progressive cognitive decline. Doi:10.1186/1752-1947-7-229 Cite this article as: Zafar et al.: A man with small vessel vasculitis presenting with brachial diplegia, multiple cranial mononeuropathies and severe orthostatic hypotension in diabetes mellitus: a case report. Authors’ contributions SFZ and EEU obtained patient history and examination, and analyzed and interpreted the patient data regarding the brachial diplegia and vasculitis and were major contributors to writing the manuscript. Author details 1Department of Neurology, Baylor College of Medicine, One Baylor Plaza, MS NB 302, Houston, TX 77030, USA. Author details 1Department of Neurology, Baylor College of Medicine, One Baylor Plaza, MS NB 302, Houston, TX 77030, USA. 2Department of Pathology and Immunology, Baylor College of Medicine - BCM 315, One Baylor Plaza, Houston, TX 77030, USA
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