Overview
Autosomal dominant slowed nerve conduction velocity is a rare inherited peripheral neuropathy classified within the spectrum of Charcot-Marie-Tooth disease type 1 (CMT1) and related demyelinating neuropathies. It is characterized by uniformly reduced motor nerve conduction velocities (typically below 38 m/s in the median nerve), indicating impaired myelin function in peripheral nerves. This condition is mapped under the broader category of hereditary motor and sensory neuropathies (HMSN) and is classified with ICD-10 code G60.0 (hereditary motor and sensory neuropathy). The disease primarily affects the peripheral nervous system, leading to progressive weakness and atrophy of distal muscles, particularly in the lower legs and feet, as well as the hands. Patients may experience sensory loss, reduced or absent deep tendon reflexes, foot deformities such as pes cavus (high-arched feet) and hammer toes, and difficulty with fine motor tasks. Onset is typically in childhood or adolescence, though severity and progression can vary considerably even within the same family. Some individuals may remain mildly affected, while others develop significant disability over time. There is currently no cure for autosomal dominant slowed nerve conduction velocity. Management is supportive and multidisciplinary, including physical therapy to maintain muscle strength and flexibility, occupational therapy for hand function, orthotic devices (such as ankle-foot orthoses) to improve gait, and surgical intervention for severe skeletal deformities when necessary. Pain management may be needed for neuropathic symptoms. Genetic counseling is recommended for affected families, as each child of an affected individual has a 50% chance of inheriting the condition.
Autosomal dominant
Passed on from just one parent; each child has about a 50% chance of inheriting it
Childhood
Begins in childhood, roughly ages 1 to 12
Treatments
No FDA-approved treatments are currently listed for Autosomal dominant slowed nerve conduction velocity.
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Specialists
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Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
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Common questions about Autosomal dominant slowed nerve conduction velocity
What is Autosomal dominant slowed nerve conduction velocity?
Autosomal dominant slowed nerve conduction velocity is a rare inherited peripheral neuropathy classified within the spectrum of Charcot-Marie-Tooth disease type 1 (CMT1) and related demyelinating neuropathies. It is characterized by uniformly reduced motor nerve conduction velocities (typically below 38 m/s in the median nerve), indicating impaired myelin function in peripheral nerves. This condition is mapped under the broader category of hereditary motor and sensory neuropathies (HMSN) and is classified with ICD-10 code G60.0 (hereditary motor and sensory neuropathy). The disease primarily
How is Autosomal dominant slowed nerve conduction velocity inherited?
Autosomal dominant slowed nerve conduction velocity follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Autosomal dominant slowed nerve conduction velocity typically begin?
Typical onset of Autosomal dominant slowed nerve conduction velocity is childhood. Age of onset can vary across affected individuals.