2009 ICD-9-CM Volume 1 Diagnosis Codes Home > Diseases Of The Nervous System And Sense Organs 320-389 > Disorders Of The Peripheral Nervous System 350-359 > ICD-9-CM Diagnosis 356 Hereditary and idiopathic peripheral neuropathy 356 is a non-specific code that cannot be used to specify a diagnosis ICD-9-CM Diagnosis 356.0 Hereditary peripheral neuropathy 356.0 is a specific code that can be used to specify a diagnosis 356.0 contains 9 index entries ICD-9-CM Diagnosis 356.1 Peroneal muscular atrophy 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) 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) 356.1 is a specific code that can be used to specify a diagnosis 356.1 contains 11 index entries ICD-9-CM Diagnosis 356.2 Hereditary sensory neuropathy 356.2 is a specific code that can be used to specify a diagnosis 356.2 contains 4 index entries ICD-9-CM Diagnosis 356.3 Refsum's disease An autosomal recessive familial disorder that usually presents in childhood with POLYNEUROPATHY, sensorineural DEAFNESS; ICHTHYOSIS; ATAXIA; RETINITIS PIGMENTOSA; and cardiomyopathy. CEREBROSPINAL FLUID PROTEINS and serum PHYTANIC ACID are generally elevated. This condition is associated with the impaired metabolism of phytanic acid in PEROXISOMES. (From Joynt, Clinical Neurology, 1991, Ch37, p58-9; Rev Med Interne 1996;17(5):391-8) 356.3 is a specific code that can be used to specify a diagnosis 356.3 contains 6 index entries ICD-9-CM Diagnosis 356.4 Idiopathic progressive polyneuropathy 356.4 is a specific code that can be used to specify a diagnosis 356.4 contains 3 index entries ICD-9-CM Diagnosis 356.8 Other specified idiopathic peripheral neuropathy 356.8 is a specific code that can be used to specify a diagnosis 356.8 contains 4 index entries ICD-9-CM Diagnosis 356.9 Unspecified idiopathic peripheral neuropathy 356.9 is a specific code that can be used to specify a diagnosis 356.9 contains 17 index entries