Cerebral palsy athetoid

Alternate names

Athetoid cerebral palsy; Cerebral palsy dyskinetic

Definition

Athetoid cerebral palsy, also known as dyskinetic cerebral palsy, is a subtype of cerebral palsy (CP). Cerebral palsy is a group of non-progressive, permanent disorders that causes limitation of activity by affecting the developing motor control circuit.

Epidemiology

• Cerebral palsy overall is the most common cause of childhood disability, occurring in 1.5 to 2.5 per 1000 live births.
• Dyskinetic or athetoid type cerebral palsy makes up 12% to 14% of the cerebral palsy cases overall.
• This calculates to around 1.8 to 3.5 cases per 10000 live births for dyskinetic cerebral palsy.

Cause

• Dyskinetic CP (DCP) is associated with basal ganglia and thalamic injuries.
• These injuries are usually seen with brief but profound hypoxic insults.
• Patients with dyskinetic CP are more commonly term infants versus those with spastic CP.
• Another common cause is neonatal hyperbilirubinemia induced kernicterus as there is bilirubin deposition in the basal ganglia.
• As kernicterus has become less common because of preventive strategies in hyperbilirubinemia, there has been a decrease in the incidence of DCP. Additionally, any insults such as intracranial hemorrhage, stroke, or cerebral infection to the basal ganglia and thalamus can result in dyskinetic CP.

Signs and symptoms

• Athetoid cerebral palsy is defined by abnormal postures and movements.
• These abnormalities are due to impaired muscle tone, impaired movement control, and impaired coordination.
• These abnormalities can be described by detailing the typical movements themselves, such as dystonic, extrapyramidal, choreoathetotic, choreoathetoid, or athetoid cerebral palsy.

Diagnosis

• Dyskinetic cerebral palsy, like all forms of cerebral palsy, is a clinical diagnosis. <ref>Li X, Arya K. Athetoid Cerebral Palsy. [Updated 2021 Jan 11]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK563160/</ref>[1].
• The history should identify risk factors for athetoid cerebral palsy, such as perinatal asphyxia or severe hyperbilirubinemia in the perinatal period.
• The physical exam is an important part of evaluating a patient with dyskinetic cerebral palsy.
• Common clinical findings in dyskinetic cerebral palsy include the following:
• In early infancy: Delayed motor development, reduced spontaneous movements, variable tone with movements while hypotonic at rest, and persistence of primitive reflexes.
• By age 2 to 3: Involuntary movements are more apparent along with abnormal posturing. This includes head being persistently turned to one side, extension in the supine position, flexion, and shoulder retraction while prone.
• Other than the clinical history and physical exam, as mentioned above, neuroimaging is helpful in the assessment of athetoid cerebral palsy.
• 70% of patients with dyskinetic cerebral palsy have lesions found on magnetic resonance imaging (MRI) of the brain in the basal ganglia or thalamus.

Treatment

• Management of dyskinetic CP centers around the management of symptoms and aims at improvement in the quality of life for the patient.
• This involves the management of dystonia and choreoathetosis, as well as helping with associated pain, disability, and discomfort.
• There is also a focus on the treatment of comorbidities that are non-motor.
• These comorbidities include epilepsy and depression.
• Other issues include ensuring optimal nutrition, possibilities of contractures, and orthopedic complications.
• There are medications that are often used in the treatment of dyskinetic cerebral palsy; however, most of them show low efficacy.
• The most commonly used drug in DCP is oral baclofen, a GABA-B agonist.
• Trihexyphenidyl is often used for dystonia. Efficacy for both of these drugs in DCP is, however, low.

Medications that are often used as an attempt to manage movement symptoms are below.

• For dystonia: dopamine agonists (levodopa), anticholinergics (trihexyphenidyl, benztropine), benzodiazepine receptor agonists (diazepam, clonazepam), GABA-B receptor agonist (baclofen), monoamine blockers (tetrabenazine), and voltage-gated sodium channel blockers (carbamazepine).
• For chorea: benzodiazepine receptor agonists (diazepam, clonazepam), dopamine antagonists (pimozide, haloperidol), monoamine blockers, and calcium channel blockers (levetiracetam).
• Deep brain stimulation (DBS) has been used increasingly in those with dyskinetic cerebral palsy. It is often used to decrease dystonia.
• Botulinum toxin is another treatment often used in the management of dystonia in DCP.<ref>Li X, Arya K. Athetoid Cerebral Palsy. [Updated 2021 Jan 11]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK563160/</ref>[2].

References

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NIH genetic and rare disease info

• NIH info on Cerebral palsy athetoid

Cerebral palsy athetoid is a rare disease.

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