Related Experiment Video
Updated: Aug 29, 2026

In Vivo Electrophysiological Measurement of Compound Muscle Action Potential from the Forelimbs in Mouse Models of Motor Neuron Degeneration
Published on: June 15, 2018
[Hereditary neuropathies]
Svein Ivar Mellgren1, Christian Vedeler
1Nevrologisk avdeling, Universitetssykehuset Nord-Norge, 9038 Tromsø. svein.ivar.mellgren@unn.no
Background:
Hereditary neuropathies constitute a heterogeneous group of diseases that make up a significant proportion of peripheral nerve disease cases.
Material And Methods:
The paper is based on a review of recent literature, including searches on Medline, and our own clinical and research experience.
Results:
Charcot-Marie-Tooth disease, itself a heterogeneous disease, is the most common hereditary neuropathy. Several variants of neuropathy are associated with hereditary metabolic disorders. Diagnostic methods include nerve conduction velocity studies and electromyography, quantitative sensory testing, molecular genetic diagnostic testing, and in selected cases processing of nerve biopsies and skin biopsies for determination of epidermal nerve fibre densities.
Interpretation:
A thorough family history of neuropathic symptoms and signs, and preferably clinical and electrophysiological examination of relatives is essential for the diagnosis of hereditary neuropathy. Molecular genetic analysis is promising for accurate classification of these diseases. Nerve biopsy is only helpful in selected cases.
Related Concept Videos
Diabetic Neuropathy
Disorders of the Nervous Tissue
Homeostatic Imbalances:
Alzheimer's disease manifests as a gradual decline in memory and cognitive abilities, attributed to the buildup of amyloid plaques and neurofibrillary tangles in the brain.
Parkinson's disease arises from the...
Huntington Disease l: Introduction
Local Anesthetics: Differential Sensitivity of Nerve Fibers
Pedigree Analysis
Pleiotropy
