Objective: To study a patient with axonal polyneuropathy due to acute dimethylamine borane (DMAB) intoxication.
Patient: Confusion and drowsiness in the acute stage, followed by cognitive impairments and polyneuropathy, are reported in a chemical factory worker after acute exposure to DMAB.
Results: Nerve conduction studies indicated axonal polyneuropathy, particularly in the motor nerves. Sural nerve biopsy studies 3 months later revealed an axonal degeneration with a mild decrease of fiber density in the large myelinated fibers. Quantitative sensory testing also disclosed an impairment of pinprick, temperature, and touch sensations. Cutaneous nerve biopsy studies 9 months later demonstrated a moderate loss of epidermal nerves. During the follow-up period of 1.5 years, the clinical features and serial nerve conduction studies showed a steady improvement.
Conclusions: Since DMAB is a new product and has been widely used recently in the manufacturing of semiconductors and electronics, we conclude that DMAB intoxication may produce motor-predominant axonal polyneuropathy and that the establishment of a threshold limit value is warranted.
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http://dx.doi.org/10.1001/archneur.63.7.1009 | DOI Listing |
Pediatr Neurol
December 2024
Genetics Research Center, University of Social Welfare and Rehabilitation Sciences, Tehran, Iran; Neuromuscular Research Center, Tehran University of Medical Sciences, Tehran, Iran. Electronic address:
Background: Spinocerebellar ataxia with axonal neuropathy 1 (SCAN1) is an ultrarare neurodegenerative disorder inherited in an autosomal recessive manner, mainly marked by progressive ataxia and axonal polyneuropathy. SCAN1 is mainly caused by the c.1478A>G:p.
View Article and Find Full Text PDFJ Neurol
January 2025
Western Institute of Neuroscience, Western University, London, Canada.
Background: Repeat neurological assessment is standard in cases of severe acute brain injury. However, conventional measures rely on overt behavior. Unfortunately, behavioral responses may be difficult or impossible for some patients.
View Article and Find Full Text PDFJ Neurol
January 2025
Department of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing, 100070, China.
Background And Objective: Neuronal intranuclear inclusion disease (NIID) is a multifaceted disorder impacting both the central and peripheral nervous systems. This study aims to investigate the clinical and electrophysiological characteristics of peripheral neuropathy in patients with NIID.
Methods: In this cross-sectional study, patients diagnosed with NIID were prospectively recruited from multiple centers across China between October 2017 and May 2024.
Cureus
December 2024
Internal Medicine, Centro Hospitalar e Universitário de Coimbra, Coimbra, PRT.
Hemophagocytic lymphohistiocytosis (HLH) is a rare and potentially fatal hyperinflammatory syndrome characterized by dysregulated immune activation and systemic inflammation. Secondary HLH is often triggered by infections, with being an infrequently reported cause. Peripheral axonal neuropathy is a rare and poorly understood complication of HLH.
View Article and Find Full Text PDFClin Neurol Neurosurg
January 2025
Department of Translational Medicine, Seoul National University College of Medicine, Seoul, Republic of Korea.
Diabetic polyneuropathy is the common neuropathy of diabetes. However, several inflammatory neuropathies may occur during diabetes. Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) represents the most treatable example.
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