Scribd Logo
Upload

Welcome to Scribd!

  • 31 views

    Ohtahara Syndrome: Corticosteroids (Prednisolone or ACTH) Are Occasionally Helpful

    Uploaded by

    idscribddotcom
    Ohtahara syndrome is a severe form of epileptic encephalopathy characterized by frequent tonic spasms within the first 3 months of life, leading to psychomotor impairment. It is caused by structural brain abnormalities, metabolic disorders, or genetic brain malformations. Symptoms include tonic seizures occurring hundreds of times per day regardless of sleep, psychomotor development delays, and neurological issues like paralysis or lack of coordination. Diagnosis involves clinical observation, EEG showing burst suppression, MRI, and metabolic screening. Treatment options include anticonvulsants, corticosteroids, ketogenic diet, surgery for focal lesions, and supportive care, but the prognosis is often progressive with death or profound handicaps.

    Copyright:

    © All Rights Reserved
    Download as DOCX, PDF, TXT or read online from Scribd

    Ohtahara Syndrome: Corticosteroids (Prednisolone or ACTH) Are Occasionally Helpful

    Uploaded by

    idscribddotcom
    31 views2 pages
    Ohtahara syndrome is a severe form of epileptic encephalopathy characterized by frequent tonic spasms within the first 3 months of life, leading to psychomotor impairment. It is caused by structural brain abnormalities, metabolic disorders, or genetic brain malformations. Symptoms include tonic seizures occurring hundreds of times per day regardless of sleep, psychomotor development delays, and neurological issues like paralysis or lack of coordination. Diagnosis involves clinical observation, EEG showing burst suppression, MRI, and metabolic screening. Treatment options include anticonvulsants, corticosteroids, ketogenic diet, surgery for focal lesions, and supportive care, but the prognosis is often progressive with death or profound handicaps.

    Original Description:

    PC-ssDM

    Original Title

    PC-ssDM

    Copyright

    © © All Rights Reserved

    Available Formats

    DOCX, PDF, TXT or read online from Scribd

    Did you find this document useful?

    Is this content inappropriate?

    Ohtahara syndrome is a severe form of epileptic encephalopathy characterized by frequent tonic spasms within the first 3 months of life, leading to psychomotor impairment. It is caused by structural brain abnormalities, metabolic disorders, or genetic brain malformations. Symptoms include tonic seizures occurring hundreds of times per day regardless of sleep, psychomotor development delays, and neurological issues like paralysis or lack of coordination. Diagnosis involves clinical observation, EEG showing burst suppression, MRI, and metabolic screening. Treatment options include anticonvulsants, corticosteroids, ketogenic diet, surgery for focal lesions, and supportive care, but the prognosis is often progressive with death or profound handicaps.

    Copyright:

    © All Rights Reserved
    Download as DOCX, PDF, TXT or read online from Scribd
    Download as docx, pdf, or txt
    31 views2 pages

    Ohtahara Syndrome: Corticosteroids (Prednisolone or ACTH) Are Occasionally Helpful

    Uploaded by

    idscribddotcom
    Ohtahara syndrome is a severe form of epileptic encephalopathy characterized by frequent tonic spasms within the first 3 months of life, leading to psychomotor impairment. It is caused by structural brain abnormalities, metabolic disorders, or genetic brain malformations. Symptoms include tonic seizures occurring hundreds of times per day regardless of sleep, psychomotor development delays, and neurological issues like paralysis or lack of coordination. Diagnosis involves clinical observation, EEG showing burst suppression, MRI, and metabolic screening. Treatment options include anticonvulsants, corticosteroids, ketogenic diet, surgery for focal lesions, and supportive care, but the prognosis is often progressive with death or profound handicaps.

    Copyright:

    © All Rights Reserved
    Download as DOCX, PDF, TXT or read online from Scribd
    Download as docx, pdf, or txt
    of 2

    OHTAHARA SYNDROME

    What is Ohtahara Syndrome ? : Early infantile epileptic encephalopathy (EIEE) or Ohtahara


    syndrome, is one of the most severe forms of age-related epileptic encephalopathies,
    characterized by the onset of tonic spasms within the first 3 months of life that can be
    generalized or lateralized, independent of the sleep cycle and that can occur hundreds of
    times per day, leading to psychomotor impairment and death

    What are the Causes ? :


    1. Structural brain abnormalities
    2. Metabolic disorders
    3. Brain malformations that may or not be genetic in origin

    What are the Symptoms ? :


    1. tonic seizures (Can appear and last up to 10 seconds, the pattern of these spasms
    remains unchanged during wakefulness and sleep and they can occur hundreds of times
    per day)

    2. psychomotor development delay (delay in development of mental and motor skills)


    3. there may development of other neurological difficulties such as:

     diplegia (paralysis of like parts on either side of the body)

     hemiplegia (paralysis/weakness of one side of the body)

     tetraplegia (spasticity and weakness of all four limbs)

     ataxia (a lack of balance and coordination)

     dystonia (abnormal movements with increased tone)

    How is it Diagnosed ? :
    1. Clinical sign

    2. EEG “burst suppression”

    3. MRI

    4. Metabolic Screening

    HOW IS IT TREATED ?
    Pharmacotherapy :
    1. Anticonvulsant drugs (such as benzodiazepines, valproate, levetiracetam, zonisamide
    and phenobarbital) - even it’s not usually very effective for this disorder.

    2. Corticosteroids (prednisolone or ACTH) are occasionally helpful


    Therapy :
    Ketogenic diet (high fat, low carbohydrate)

    Treatment for selected patients :


    In cases where there is a focal brain lesion (damage or abnormal development of one
    area/side of the brain), surgery may be beneficial.

    Other Treatments :
    They are symptomatic and supportive; these can include tratments for abnormal muscle
    tone, irritability, or stomach or lung problems.

    Complication
    Chest infection : Bronchitis, Pneumonia

    TAMBAHAN JIKA CUKUP

    Prognosis
    The course of Ohtahara syndrome is severely progressive. Seizures become more frequent,
    accompanied by delays in physical and cognitive development. Some children will die in
    infancy; others will survive but be profoundly handicapped. As they grow, some children will
    progress into other epilepsy syndromes.

    You might also like