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ICD Hub / Diseases of the nervous system (G00–G99) / 8A00.21 - Hemiparkinsonism hemiatrophy syndrome
ICD-11

ICD-11 Codes
for 8A00.21 - Hemiparkinsonism hemiatrophy syndrome
Teodor Jurukovski January 1, 1970
Fact checked by: Teodor Jurukovski
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Understanding Hemiparkinsonism-Hemiatrophy Syndrome (ICD-11 Code 8A00.21)

Hemiparkinsonism-hemiatrophy syndrome, identified by ICD-11 code 8A00.21, is a rare neurological disorder. It is characterised by the presence of unilateral body atrophy alongside slowly progressive, ipsilateral hemiparkinsonian signs. These signs typically include bradykinesia (slowness of movement), rigidity, and tremor. Patients may initially present with unilateral dystonia, often action-induced and affecting the limbs, which can later become bilateral. Alternatively, a tremor predominantly occurring at rest may progress to hemiparkinsonism. This condition is also commonly referred to by synonyms such as HP-HA syndrome. It is considered a form of secondary parkinsonism, affecting fewer than 1,000 individuals in the US. Symptoms can manifest in adolescence or adulthood, and the condition is known for its clinical heterogeneity.

Recognising the Signs: Symptoms of HP-HA Syndrome

The hallmark symptoms of Hemiparkinsonism-hemiatrophy syndrome (HP-HA syndrome) involve the asymmetric presentation of neurological deficits and physical wasting. Key features include unilateral body atrophy, meaning one side of the body shows reduced muscle mass and size. This is accompanied by ipsilateral parkinsonian symptoms, meaning they occur on the same side as the atrophy. These parkinsonian signs encompass bradykinesia, rigidity, and resting tremor. Patients may also experience unilateral dystonia, which are involuntary muscle contractions causing abnormal postures, often triggered by voluntary actions. Associated clinical findings frequently observed include scoliosis (curvature of the spine), scapular winging, raised shoulders, hyperactive deep tendon reflexes, and extensor plantar responses. Other reported symptoms can include gait disturbances, dysarthria (difficulty speaking), and depression.

Potential Origins and Contributing Factors

The exact cause of Hemiparkinsonism-hemiatrophy syndrome (HP-HA syndrome) is not fully understood, but it is believed to stem from genetic mutations or changes in DNA. These genetic alterations can be inherited or occur spontaneously. In some cases, the syndrome may arise following early childhood cerebral insults. Major risk factors identified include abnormal birth histories, such as perinatal hypoxia or dystocia, as well as head trauma sustained in early childhood. Fever during infancy has also been implicated. Given these associations, HP-HA syndrome is classified as a type of secondary parkinsonism, indicating it results from a known underlying cause or structural lesion rather than being idiopathic.

Diagnostic Pathways for Hemiparkinsonism-Hemiatrophy Syndrome

Diagnosing Hemiparkinsonism-hemiatrophy syndrome (HP-HA syndrome) typically involves a comprehensive approach. This includes a detailed medical history, assessment of family history, a thorough clinical examination, and various paraclinical tests. It is noted that obtaining an accurate diagnosis can often be a lengthy process, sometimes taking over six years, and may require consultations with multiple specialists. Advanced imaging techniques such as Magnetic Resonance Imaging (MRI) and Positron Emission Tomography (PET) scans can be valuable tools. MRI may reveal findings like cerebral atrophy or lesions in specific brain areas, while PET scans, particularly FDOPA-PET and FDG-PET, can help assess dopaminergic neuron function and overall neuronal activity, aiding in understanding the condition's pathogenesis.

Managing Hemiparkinsonism-Hemiatrophy Syndrome: Treatment Approaches

Management of Hemiparkinsonism-hemiatrophy syndrome (HP-HA syndrome) focuses on alleviating symptoms, as treatment responses can be variable. Medications such as levodopa and dopamine agonists have shown mixed results, with some patients experiencing good responses while others have moderate or poor outcomes. The addition of a dopamine agonist to levodopa therapy has been reported to be beneficial in some cases. For individuals with severe symptoms, surgical interventions like deep brain stimulation (DBS) may be considered. Given the complexity and multi-systemic nature of the condition, establishing a multidisciplinary healthcare team is crucial for coordinated care and symptom management.

Documentation and Coding for ICD-11 Code 8A00.21

Accurate medical documentation and coding are essential for patient care and billing. The ICD-11 code for Hemiparkinsonism hemiatrophy syndrome is 8A00.21. This specific code is used to identify the condition, which is officially named Hemiparkinsonism hemiatrophy syndrome. Common clinical synonyms include HP-HA syndrome. When documenting this condition, healthcare providers should note the presence of unilateral body atrophy, ipsilateral parkinsonian signs (bradykinesia, rigidity, tremor), and any associated dystonia or other neurological findings. This precise coding ensures appropriate classification within medical records and facilitates accurate billing processes for healthcare services rendered.

Frequently asked questions

What is the ICD-11 classification meaning of code 8A00.21?
ICD-11 code 8A00.21 represents Hemiparkinsonism hemiatrophy syndrome, classified under Secondary parkinsonism.
How is Hemiparkinsonism hemiatrophy syndrome categorized within the ICD-11 classification?
Hemiparkinsonism hemiatrophy syndrome is categorized as a specific type of Secondary parkinsonism under the ICD-11 code 8A00.21.
What is the ICD-11 code for Hemiparkinsonism hemiatrophy syndrome?
The ICD-11 code for Hemiparkinsonism hemiatrophy syndrome is 8A00.21.
What is the parent classification for ICD-11 code 8A00.21?
The parent classification for ICD-11 code 8A00.21 (Hemiparkinsonism hemiatrophy syndrome) is Secondary parkinsonism (8A00.2).
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