icd 10 code for ohtahara syndrome

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What is Ohtahara syndrome (OS)?

Ohtahara syndrome (OS), also known as Early Infantile Epileptic Encephalopathy with Burst-Suppression (EIEE), is a progressive epileptic encephalopathy. The syndrome is outwardly characterized by tonic spasms and partial seizures, and receives its more elaborate name from the pattern of burst activity on an electroencephalogram (EEG).

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R03.0 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes. The 2018/2019 edition of ICD-10-CM R03.0 became effective on October 1, 2018.

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Q87.19 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes. Short description: Other congen malform synd predom assoc with short stature The 2021 edition of ICD-10-CM Q87.19 became effective on October 1, 2020.

What is the ICD 10 code for white coat syndrome?

White coat syndrome ICD-10-CM R03.0 is grouped within Diagnostic Related Group (s) (MS-DRG v38.0): 314 Other circulatory system diagnoses with mcc 315 Other circulatory system diagnoses with cc

What is ohtahara syndrome?

Definition. Ohtahara syndrome is an uncommon type of epilepsy characterized by hard to control seizures and developmental delays. The disorder affects infants, usually within the first three months of life (most often within the first 10 days) in the form of epileptic seizures.

How is ohtahara syndrome diagnosed?

The EEG is the most important test in making a diagnosis of Ohtahara syndrome. The EEG is very abnormal with a burst suppression pattern (high amplitude spikes followed by little brain activity or flattening of the brain waves).

What is early infantile epileptic encephalopathy?

Early Infantile Epileptic Encephalopathy (EIEE) is a neurological disorder characterized by seizures. The disorder affects newborns, usually within the first three months of life in the form of epileptic seizures.

What causes early infantile epileptic encephalopathy?

EIEE can be caused by mutations in the ARX gene. This is a regulatory gene involved in brain development, and it is thought that a reduction in the protein produced by the ARX gene is the main effect of the ARX mutation. Changes in genes not located on the X chromosome can also cause infantile spasms in rare cases.

Is ohtahara syndrome rare?

This is a very rare epilepsy syndrome. Seizures start before 3 months of age. Many babies have an underlying structural brain abnormality or a metabolic (biochemical) disorder.

Is ohtahara syndrome curable?

There are several treatment options used to manage Ohtahara syndrome, but there is not a cure. These treatments can help reduce the frequency and severity of the seizures, but they are not effective in managing developmental problems.

What is developmental and epileptic encephalopathy?

Developmental and epileptic encephalopathy 1 (DEE1) is a seizure disorder characterized by a type of seizure known as infantile spasms. The spasms usually appear before the age of 1.

What is the difference between infantile spasms and seizures?

Spasms are typically shorter than what most people think of when they think of seizures — namely, a tonic-clonic (grand mal) seizure. Infantile spasms last around one to two seconds in a series; whereas other types of seizures can last from 30 seconds to two minutes.

What causes epileptic encephalopathy?

The most common causes of epileptic encephalopathy in infancy are structural abnormalities, either congenital (such as malformations of cortical development) or acquired (such as hypoxic-ischemic insults).

How is epileptic encephalopathy diagnosed?

The diagnosis of these epileptic encephalopathies begins with an EEG which should include both the sleep and wake states. A magnetic resonance imaging of the brain must be obtained to look for structural defects.

Is epileptic encephalopathy curable?

Hormonal and immune therapies are at the forefront of treatment in many cases, with traditional antiepileptic drugs and surgery (when an identifiable lesion is present) playing a limited role. However, gold standard evidence for treatment of epileptic encephalopathies remains limited.

Is epileptic encephalopathy genetic?

Early infantile epileptic encephalopathy is genetically heterogeneous; in addition to STXBP1 and ARX, we will highlight other genes with their associated clinical presentation (e.g. cyclin-dependent kinase-like 5 (CDKL5) and solute carrier family 25 member 22 (SLC25A22)) in other sections of this guide.

What is the ICD code for Ohtahara syndrome?

The ICD code G404 is used to code Ohtahara syndrome. Ohtahara syndrome (OS), also known as Early Infantile Epileptic Encephalopathy with Burst-Suppression (EIEE), is a progressive epileptic encephalopathy. The syndrome is outwardly characterized by tonic spasms and partial seizures, and receives its more elaborate name from the pattern ...

What is the ICD code for acute care?

G40.40. Non-Billable means the code is not sufficient justification for admission to an acute care hospital when used a principal diagnosis. Use a child code to capture more detail. ICD Code G40.40 is a non-billable code.

What is the ICD code for epilepsy?

ICD Code G40.40 is a non-billable code. To code a diagnosis of this type, you must use one of the two child codes of G40.40 that describes the diagnosis 'oth generalized epilepsy, not intractable' in more detail. G40.40 Other generalized epilepsy and epileptic syndromes, not intractable.

When does Ohtahara syndrome occur?

Ohtahara syndrome is rare and the earliest-appearing age-related epileptic encephalopathy, with seizure onset occurring within the first three months of life, and often in the first ten days. Many, but not all, cases of OS evolve into other seizure disorders, namely West syndrome and Lennox-Gastaut syndrome.

What genes are associated with Ohtahara syndrome?

It can be associated with mutations in ARX, CDKL5, SLC25A22, STXBP1, SPTAN1, KCNQ2, ARHGEF9, PCDH19, PNKP, SCN2A, PLCB1, SCN8A, ST3GAL3, TBC1D24, BRAT1 and likely others .

What is the name of the syndrome that causes tonic spasms?

Ohtahara syndrome (' OS), also known as early infantile epileptic encephalopathy (EIEE ’) is a progressive epileptic encephalopathy. The syndrome is outwardly characterized by tonic spasms and partial seizures within the first few months of life, and receives its more elaborate name from the pattern of burst activity on an electroencephalogram ...

What is the name of the epileptic syndrome that is characterized by tonic spasms and partial seizures

Ohtahara syndrome. Ohtahara syndrome (' OS), also known as early infantile epileptic encephalopathy (EIEE ’) is a progressive epileptic encephalopathy. The syndrome is outwardly characterized by tonic spasms and partial seizures within the first few months of life, and receives its more elaborate name from the pattern of burst activity on an ...

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Clinical description

Onset of EIEE occurs within the first 3 months of life but some present within the first few weeks after birth. Neonates have poor suckling reflexes, hypotonia and manifest with generalized and symmetrical tonic spasms that can appear in clusters or singly and can last for up to 10 seconds.

Etiology

EIEE may be the result of different etiologies. Many cases have been associated with structural brain abnormalities.

Diagnostic methods

Diagnosis is based on clinical and electroencephalographic findings. The characteristic electroencephalogram (EEG) displays a suppression burst pattern (which appears with the onset of spasms) that is comprised of bursts of high amplitude spikes and polyspikes that alternate with periods of low voltage basic rhythm (suppression).

Differential diagnosis

Differential diagnoses include other epileptic encephalopathies such as early myoclonic encephalopathy, West syndrome (see these terms) and other early onset epileptic encephalopathies.

Antenatal diagnosis

Prenatal diagnosis is possible in families with a known genetic etiology.

Genetic counseling

Autosomal recessive inheritance has been reported, but most cases of EIEE are sporadic ( de novo mutations). Genetic counseling is therefore very valuable to inform parents that their risk of having further children with EIEE is low.