Hereditary Motor and Sensory Neuropathies
| Definition: |
A group of slowly progressive inherited disorders affecting motor and sensory peripheral nerves. Subtypes include HMSNs I-VII. HMSN I and II both refer to CHARCOT-MARIE-TOOTH DISEASE. HMSN III refers to hypertrophic neuropathy of infancy, HMSN IV refers to REFSUM DISEASE, HMSN V refers to a condition marked by a hereditary motor and sensory neuropathy associated with spastic paraplegia (see SPASTIC PARAPLEGIA, HEREDITARY). HMSN VI refers to HMSN associated with an inherited optic atrophy (OPTIC ATROPHIES, HEREDITARY), and HMSN VII refers to HMSN associated with retinitis pigmentosa. (From Adams et al., Principles of Neurology, 6th ed, p1343) |
| Notes: |
do not confuse with HEREDITARY MOTOR AND AUTONOMIC NEUROPATHIES; DF: NEUROPATHIES HEREDITARY MOTOR SENSORY |
| Also Called: |
Hereditary, Type VII, Motor and Sensory Neuropathy,HMSN Type III |
| Previously Indexed: |
Charcot-Marie Disease (1975-1988),Muscular Atrophy (1972-1988),Neuromuscular Diseases (1979-1988) |
Hereditary Motor and Sensory Neuropathies Categories.
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Charcot-Marie-Tooth Disease - A hereditary motor and sensory neuropathy transmitted most often as an autosomal dominant trait and characterized by progressive distal wasting and loss of reflexes in the muscles of the legs (and occasionally involving the arms). Onset is usually in the second to fourth decade of life. This condition has been divided into two subtypes, hereditary motor and sensory neuropathy (HMSN) types I and II. HMSN I is associated with abnormal nerve conduction velocities and nerve hypertrophy, features not seen in HMSN II. (Adams et al., Principles of Neurology, 6th ed, p1343) |
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Spastic Paraplegia, Hereditary - A group of inherited diseases that share similar phenotypes but are genetically diverse. Different genetic loci for autosomal recessive, autosomal dominant, and x-linked forms of hereditary spastic paraplegia have been identified. Clinically, patients present with slowly progessive distal limb weakness and lower extremity spasticity. Peripheral sensory neurons may be affected in the later stages of the disease. (J Neurol Neurosurg Psychiatry 1998 Jan;64(1):61-6; Curr Opin Neurol 1997 Aug;10(4):313-8) |
Hereditary Motor and Sensory Neuropathies Definitions and Terms
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