ICD-10 Code G11.4

Hereditary spastic paraplegia

Version 2019 Billable Code

Valid for Submission

G11.4 is a billable code used to specify a medical diagnosis of hereditary spastic paraplegia. The code is valid for the year 2020 for the submission of HIPAA-covered transactions.

ICD-10: G11.4
Short Description:Hereditary spastic paraplegia
Long Description:Hereditary spastic paraplegia

Code Classification

  • Diseases of the nervous system (G00–G99)
    • Systemic atrophies primarily affecting the central nervous system (G10-G14)
      • Hereditary ataxia (G11)

Code History

  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016
    (first year ICD-10-CM implemented into the HIPAA mandated code set)
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020

Information for Medical Professionals

Convert G11.4 to ICD-9

The following crosswalk between ICD-10 to ICD-9 is based based on the General Equivalence Mappings (GEMS) information:

  • 334.1 - Hered spastic paraplegia (Approximate Flag)

Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

  • Autosomal dominant hereditary spastic paraplegia
  • Autosomal dominant spastic paraplegia type 10
  • Autosomal dominant spastic paraplegia type 12
  • Autosomal dominant spastic paraplegia type 17
  • Autosomal dominant spastic paraplegia type 19
  • Autosomal dominant spastic paraplegia type 29
  • Autosomal dominant spastic paraplegia type 31
  • Autosomal dominant spastic paraplegia type 36
  • Autosomal dominant spastic paraplegia type 37
  • Autosomal dominant spastic paraplegia type 4
  • Autosomal dominant spastic paraplegia type 41
  • Autosomal dominant spastic paraplegia type 42
  • Autosomal dominant spastic paraplegia type 6
  • Autosomal recessive cerebellar ataxia with late-onset spasticity
  • Autosomal recessive spastic ataxia of Charlevoix-Saguenay
  • Autosomal recessive spastic paraplegia type 11
  • Autosomal recessive spastic paraplegia type 15
  • Autosomal recessive spastic paraplegia type 18
  • Autosomal recessive spastic paraplegia type 21
  • Autosomal recessive spastic paraplegia type 23
  • Autosomal recessive spastic paraplegia type 25
  • Autosomal recessive spastic paraplegia type 26
  • Autosomal recessive spastic paraplegia type 27
  • Autosomal recessive spastic paraplegia type 28
  • Autosomal recessive spastic paraplegia type 32
  • Autosomal recessive spastic paraplegia type 35
  • Autosomal recessive spastic paraplegia type 39
  • Autosomal recessive spastic paraplegia type 43
  • Autosomal recessive spastic paraplegia type 44
  • Autosomal recessive spastic paraplegia type 45
  • Autosomal recessive spastic paraplegia type 46
  • Autosomal recessive spastic paraplegia type 48
  • Autosomal recessive spastic paraplegia type 53
  • Autosomal recessive spastic paraplegia type 54
  • Autosomal recessive spastic paraplegia type 55
  • Autosomal recessive spastic paraplegia type 57
  • Autosomal recessive spastic paraplegia type 58
  • Autosomal recessive spastic paraplegia type 59
  • Autosomal recessive spastic paraplegia type 5A
  • Autosomal recessive spastic paraplegia type 61
  • Autosomal recessive spastic paraplegia type 62
  • Autosomal recessive spastic paraplegia type 63
  • Autosomal recessive spastic paraplegia type 64
  • Autosomal recessive spastic paraplegia type 67
  • Autosomal recessive spastic paraplegia type 70
  • Autosomal spastic paraplegia type 30
  • Bedouin spastic ataxia syndrome
  • Choreoathetosis
  • Chronic myelopathy
  • Complicated hereditary spastic paraplegia
  • Congenital atrophy of optic nerve
  • Congenital miosis
  • Early-onset spastic ataxia, myoclonic epilepsy, neuropathy syndrome
  • Fryns macrocephaly
  • Hereditary degenerative disease of central nervous system
  • Hereditary sensory and autonomic neuropathy with spastic paraplegia
  • Hereditary spastic paraplegia
  • Infantile ascending hereditary spastic paralysis
  • Inherited spastic paresis
  • Macular corneal dystrophy
  • MT-ATP6-related mitochondrial spastic paraplegia
  • Paresis of lower extremity
  • Parkinsonism due to hereditary spastic paraplegia
  • Paroxysmal choreoathetosis
  • Paroxysmal dystonia
  • Paroxysmal dystonic choreoathetosis with episodic ataxia and spasticity
  • Pure hereditary spastic paraplegia
  • Severe intellectual disability
  • Severe intellectual disability and progressive spastic paraplegia
  • Spastic ataxia with congenital miosis
  • Spastic paraparesis
  • Spastic paraparesis and deafness
  • Spastic paraplegia
  • Spastic paraplegia type 7
  • Spastic paraplegia with Paget disease of bone syndrome
  • Spastic paraplegia with precocious puberty syndrome
  • Spastic paraplegia, facial cutaneous lesion syndrome
  • Spastic paraplegia, glaucoma, intellectual disability syndrome
  • Spastic paraplegia, nephritis, deafness syndrome
  • Spastic paraplegia, neuropathy, poikiloderma syndrome
  • Spastic paraplegia, optic atrophy, neuropathy syndrome
  • X-linked hereditary spastic paraplegia
  • X-linked spastic paraplegia type 2
  • X-linked spastic paraplegia type 34

Index to Diseases and Injuries

The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10 code(s). The following references for the code G11.4 are found in the index:


Information for Patients


Neuromuscular Disorders

Neuromuscular disorders affect your neuromuscular system. They can cause problems with

  • The nerves that control your muscles
  • Your muscles
  • Communication between your nerves and muscles

These disorders can cause your muscles to become weak and waste away. You may also have symptoms such as spasms, twitching, and pain.

Examples of neuromuscular disorders include

  • Amyotrophic lateral sclerosis
  • Muscular dystrophy
  • Myasthenia gravis
  • Spinal muscular atrophy

There can be different causes for these diseases. Many of them are genetic.This means they are inherited (run in families) or are caused by a new mutation in your genes. Some neuromuscular disorders are autoimmune diseases. Sometimes the cause is not known.

Many neuromuscular diseases have no cure. But treatments may improve symptoms, increase mobility, and lengthen life.


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Paralysis

Paralysis is the loss of muscle function in part of your body. It happens when something goes wrong with the way messages pass between your brain and muscles. Paralysis can be complete or partial. It can occur on one or both sides of your body. It can also occur in just one area, or it can be widespread. Paralysis of the lower half of your body, including both legs, is called paraplegia. Paralysis of the arms and legs is quadriplegia.

Most paralysis is due to strokes or injuries such as spinal cord injury or a broken neck. Other causes of paralysis include

  • Nerve diseases such as amyotrophic lateral sclerosis
  • Autoimmune diseases such as Guillain-Barre syndrome
  • Bell's palsy, which affects muscles in the face

Polio used to be a cause of paralysis, but polio no longer occurs in the U.S.


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Silver syndrome Silver syndrome belongs to a group of genetic disorders known as hereditary spastic paraplegias. These disorders are characterized by progressive muscle stiffness (spasticity) and, frequently, development of paralysis of the lower limbs (paraplegia). Hereditary spastic paraplegias are divided into two types: pure and complex. Both types involve the lower limbs; the complex types may also involve the upper limbs, although to a lesser degree. In addition, the complex types may affect the brain and parts of the nervous system involved in muscle movement and sensations. Silver syndrome is a complex hereditary spastic paraplegia.The first sign of Silver syndrome is usually weakness in the muscles of the hands. These muscles waste away (amyotrophy), resulting in abnormal positioning of the thumbs and difficulty using the fingers and hands for tasks such as handwriting. People with Silver syndrome often have high-arched feet (pes cavus) and spasticity in the legs. The signs and symptoms of Silver syndrome typically begin in late childhood but can start anytime from early childhood to late adulthood. The muscle problems associated with Silver syndrome slowly worsen with age, but affected individuals can remain active throughout life.
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Spastic paraplegia type 11 Spastic paraplegia type 11 is part of a group of genetic disorders known as hereditary spastic paraplegias. These disorders are characterized by progressive muscle stiffness (spasticity) and the development of paralysis of the lower limbs (paraplegia). Hereditary spastic paraplegias are divided into two types: pure and complex. The pure types involve the lower limbs. The complex types involve the lower limbs and can affect the upper limbs to a lesser degree. Complex spastic paraplegias also affect the structure or functioning of the brain and the peripheral nervous system, which consists of nerves connecting the brain and spinal cord to muscles and sensory cells that detect sensations such as touch, pain, heat, and sound. Spastic paraplegia type 11 is a complex hereditary spastic paraplegia.Like all hereditary spastic paraplegias, spastic paraplegia type 11 involves spasticity of the leg muscles and muscle weakness. In almost all individuals with this type of spastic paraplegia, the tissue connecting the left and right halves of the brain (corpus callosum) is abnormally thin. People with this form of spastic paraplegia can also experience numbness, tingling, or pain in the arms and legs (sensory neuropathy); disturbance in the nerves used for muscle movement (motor neuropathy); intellectual disability; exaggerated reflexes (hyperreflexia) of the lower limbs; speech difficulties (dysarthria); reduced bladder control; and muscle wasting (amyotrophy). Less common features include difficulty swallowing (dysphagia), high-arched feet (pes cavus), an abnormal curvature of the spine (scoliosis), and involuntary movements of the eyes (nystagmus). The onset of symptoms varies greatly; however, abnormalities in muscle tone and difficulty walking usually become noticeable in adolescence.Many features of spastic paraplegia type 11 are progressive. Most people experience a decline in intellectual ability and an increase in muscle weakness and nerve abnormalities over time. As the condition progresses, some people require wheelchair assistance.
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Spastic paraplegia type 15 Spastic paraplegia type 15 is part of a group of genetic disorders known as hereditary spastic paraplegias. These disorders are characterized by progressive muscle stiffness (spasticity) and the development of paralysis of the lower limbs (paraplegia). Spastic paraplegia type 15 is classified as a complex hereditary spastic paraplegia because it involves all four limbs as well as additional features, including abnormalities of the brain. In addition to the muscles and brain, spastic paraplegia type 15 affects the peripheral nervous system, which consists of nerves connecting the brain and spinal cord to muscles and sensory cells that detect sensations such as touch, pain, heat, and sound.Spastic paraplegia type 15 usually becomes apparent in childhood or adolescence with the development of weak muscle tone (hypotonia), difficulty walking, or intellectual disability. In almost all affected individuals, the tissue connecting the left and right halves of the brain (corpus callosum) is abnormally thin and becomes thinner over time. Additionally, there is often a loss (atrophy) of nerve cells in several parts of the brain, including the cerebral cortex, which controls thinking and emotions, and the cerebellum, which coordinates movement.People with this form of spastic paraplegia can have numbness, tingling, or pain in the arms and legs (sensory neuropathy); impairment of the nerves used for muscle movement (motor neuropathy); exaggerated reflexes (hyperreflexia) of the lower limbs; muscle wasting (amyotrophy); or reduced bladder control. Rarely, spastic paraplegia type 15 is associated with a group of movement abnormalities called parkinsonism, which includes tremors, rigidity, and unusually slow movement (bradykinesia). People with spastic paraplegia type 15 may have an eye condition called pigmentary maculopathy that often impairs vision. This condition results from the breakdown (degeneration) of tissue at the back of the eye called the macula, which is responsible for sharp central vision.Most people with spastic paraplegia type 15 experience a decline in intellectual ability and an increase in muscle weakness and nerve abnormalities over time. As the condition progresses, many people require walking aids or wheelchair assistance in adulthood.
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Spastic paraplegia type 2 Spastic paraplegia type 2 is part of a group of genetic disorders known as hereditary spastic paraplegias. These disorders are characterized by progressive muscle stiffness (spasticity) and the development of paralysis of the lower limbs (paraplegia). Hereditary spastic paraplegias are divided into two types: pure and complex. The pure types involve the lower limbs. The complex types involve the lower limbs and can also affect the upper limbs to a lesser degree; the structure or functioning of the brain; and the nerves connecting the brain and spinal cord to muscles and sensory cells that detect sensations such as touch, pain, heat, and sound (the peripheral nervous system). Spastic paraplegia type 2 can occur in either the pure or complex form.People with the pure form of spastic paraplegia type 2 experience spasticity in the lower limbs, usually without any additional features. People with the complex form of spastic paraplegia type 2 have lower limb spasticity and can also experience problems with movement and balance (ataxia); involuntary movements of the eyes (nystagmus); mild intellectual disability; involuntary, rhythmic shaking (tremor); and degeneration (atrophy) of the optic nerves, which carry information from the eyes to the brain. Symptoms usually become apparent between the ages of 1 and 5 years; those affected are typically able to walk and have a normal lifespan.
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Spastic paraplegia type 31 Spastic paraplegia type 31 is one of a group of genetic disorders known as hereditary spastic paraplegias. These disorders are characterized by progressive muscle stiffness (spasticity) and the development of paralysis of the lower limbs (paraplegia) caused by degeneration of nerve cells (neurons) that trigger muscle movement. Hereditary spastic paraplegias are divided into two types: pure and complicated. The pure types involve only the lower limbs, while the complicated types also involve the upper limbs and other areas of the body, including the brain. Spastic paraplegia type 31 is usually a pure hereditary spastic paraplegia, although a few complicated cases have been reported.The first signs and symptoms of spastic paraplegia type 31 usually appear before age 20 or after age 30. An early feature is difficulty walking due to spasticity and weakness, which typically affect both legs equally. People with spastic paraplegia type 31 can also experience progressive muscle wasting (amyotrophy) in the lower limbs, exaggerated reflexes (hyperreflexia), a decreased ability to feel vibrations, reduced bladder control, and high-arched feet (pes cavus). As the condition progresses, some individuals require walking support.
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Spastic paraplegia type 3A Spastic paraplegia type 3A is one of a group of genetic disorders known as hereditary spastic paraplegias. These disorders are characterized by muscle stiffness (spasticity) and weakness in the lower limbs (paraplegia). Hereditary spastic paraplegias are often divided into two types: pure and complex. The pure types involve only the lower limbs, while the complex types also involve other areas of the body; additional features can include changes in vision, changes in intellectual functioning, difficulty walking, and disturbances in nerve function (neuropathy). Spastic paraplegia type 3A is usually a pure hereditary spastic paraplegia, although a few complex cases have been reported.In addition to spasticity and weakness, which typically affect both legs equally, people with spastic paraplegia type 3A can also experience progressive muscle wasting (amyotrophy) in the lower limbs, reduced bladder control, an abnormal curvature of the spine (scoliosis), loss of sensation in the feet (peripheral neuropathy), or high arches of the feet (pes cavus). The signs and symptoms of spastic paraplegia type 3A usually appear before the age of 10; the average age of onset is 4 years. In some affected individuals the condition slowly worsens over time, sometimes leading to a need for walking support.
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Spastic paraplegia type 4 Spastic paraplegia type 4 is part of a group of genetic disorders known as hereditary spastic paraplegias. These disorders are characterized by progressive muscle stiffness (spasticity) and the development of paralysis of the lower limbs (paraplegia). Hereditary spastic paraplegias are divided into two types: pure and complex. The pure types involve only the lower limbs, whereas the complex types also involve the upper limbs (to a lesser degree) and the nervous system. Spastic paraplegia type 4 is a pure hereditary spastic paraplegia.Like all hereditary spastic paraplegias, spastic paraplegia type 4 involves spasticity of the leg muscles and muscle weakness. People with this condition can also experience exaggerated reflexes (hyperreflexia), ankle spasms, high-arched feet (pes cavus), and reduced bladder control. Spastic paraplegia type 4 generally affects nerve and muscle function in the lower half of the body only.
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Spastic paraplegia type 5A Spastic paraplegia type 5A is one of a group of genetic disorders known as hereditary spastic paraplegias. These disorders are characterized by muscle stiffness (spasticity) and severe weakness in the lower limbs (paraplegia). Hereditary spastic paraplegias are often divided into two types: pure and complex. The pure types involve spasticity and weakness only in the lower limbs, while the complex types involve additional problems with other areas of the body; additional features can include changes in vision, changes in intellectual functioning, brain abnormalities, and disturbances in nerve function (neuropathy). Spastic paraplegia type 5A is usually a pure hereditary spastic paraplegia, although complex type features have been reported in some individuals, usually in those who have had the condition for many years.In addition to spasticity and weakness, people with spastic paraplegia type 5A can lose the ability to sense the position of their limbs or detect vibrations with their lower limbs. They may also have muscle wasting (amyotrophy), reduced bladder control, or high arches of the feet (pes cavus). The signs and symptoms of spastic paraplegia type 5A usually appear in adolescence but can begin at any time between infancy and mid-adulthood. The condition slowly worsens over time, often leading affected individuals to require walking support or wheelchair assistance.
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Spastic paraplegia type 7 Spastic paraplegia type 7 is part of a group of genetic disorders known as hereditary spastic paraplegias. These disorders are characterized by progressive muscle stiffness (spasticity) and the development of paralysis of the lower limbs (paraplegia). Hereditary spastic paraplegias are divided into two types: pure and complex. The pure types involve the lower limbs. The complex types involve the lower limbs and can also affect the upper limbs to a lesser degree; the structure or functioning of the brain; and the nerves connecting the brain and spinal cord to muscles and sensory cells that detect sensations such as touch, pain, heat, and sound (the peripheral nervous system). Spastic paraplegia type 7 can occur in either the pure or complex form.Like all hereditary spastic paraplegias, spastic paraplegia type 7 involves spasticity of the leg muscles and increased muscle weakness. People with this form of spastic paraplegia can also experience exaggerated reflexes (hyperreflexia) in the arms; speech difficulties (dysarthria); difficulty swallowing (dysphagia); involuntary movements of the eyes (nystagmus); mild hearing loss; abnormal curvature of the spine (scoliosis); high-arched feet (pes cavus); numbness, tingling, or pain in the arms and legs (sensory neuropathy); disturbance in the nerves used for muscle movement (motor neuropathy); and muscle wasting (amyotrophy). The onset of symptoms varies greatly among those with spastic paraplegia type 7; however, abnormalities in muscle tone and other features are usually noticeable in adulthood.
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Spastic paraplegia type 8 Spastic paraplegia type 8 is part of a group of genetic disorders known as hereditary spastic paraplegias. These disorders are characterized by progressive muscle stiffness (spasticity) and the development of paralysis of the lower limbs (paraplegia). Hereditary spastic paraplegias are divided into two types: pure and complex. The pure types involve only the nerves and muscles controlling the lower limbs and bladder, whereas the complex types also have significant involvement of the nervous system in other parts of the body. Spastic paraplegia type 8 is a pure hereditary spastic paraplegia.Like all hereditary spastic paraplegias, spastic paraplegia type 8 involves spasticity of the leg muscles and muscle weakness. People with this condition can also experience exaggerated reflexes (hyperreflexia), a decreased ability to feel vibrations, muscle wasting (amyotrophy), and reduced bladder control. The signs and symptoms of spastic paraplegia type 8 usually appear in early to mid-adulthood. As the muscle weakness and spasticity get worse, some people may need the aid of a cane, walker, or wheelchair.
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Troyer syndrome Troyer syndrome is part of a group of genetic disorders known as hereditary spastic paraplegias. These disorders are characterized by progressive muscle stiffness (spasticity) and the development of paralysis of the lower limbs (paraplegia). Hereditary spastic paraplegias are divided into two types: pure and complex. The pure types involve the lower limbs. The complex types involve the lower limbs and can also affect the upper limbs to a lesser degree; the structure or functioning of the brain; and the nerves connecting the brain and spinal cord to muscles and sensory cells that detect sensations such as touch, pain, heat, and sound (the peripheral nervous system). Troyer syndrome is a complex hereditary spastic paraplegia.People with Troyer syndrome can experience a variety of signs and symptoms. The most common characteristics of Troyer syndrome are spasticity of the leg muscles, progressive muscle weakness, paraplegia, muscle wasting in the hands and feet (distal amyotrophy), small stature, developmental delay, learning disorders, speech difficulties (dysarthria), and mood swings. Other characteristics can include exaggerated reflexes (hyperreflexia) in the lower limbs, uncontrollable movements of the limbs (choreoathetosis), skeletal abnormalities, and a bending outward (valgus) of the knees.Troyer syndrome causes the degeneration and death of muscle cells and motor neurons (specialized nerve cells that control muscle movement) throughout a person's lifetime, leading to a slow progressive decline in muscle and nerve function. The severity of impairment related to Troyer syndrome increases as a person ages. Most affected individuals require a wheelchair by the time they are in their fifties or sixties.
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ICD-10 Footnotes

General Equivalence Map Definitions
The ICD-10 and ICD-9 GEMs are used to facilitate linking between the diagnosis codes in ICD-9-CM and the new ICD-10-CM code set. The GEMs are the raw material from which providers, health information vendors and payers can derive specific applied mappings to meet their needs.

  • Approximate Flag - The approximate flag is on, indicating that the relationship between the code in the source system and the code in the target system is an approximate equivalent.
  • No Map Flag - The no map flag indicates that a code in the source system is not linked to any code in the target system.
  • Combination Flag - The combination flag indicates that more than one code in the target system is required to satisfy the full equivalent meaning of a code in the source system.