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| Name | Class |
|---|---|
| National Institute of Child Health, Karachi, Pakistan | OTHER_GOV |
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SSPE is a progressive neurodegenerative disorder occurring after measles infection and is potentially life-threatening.Measles infection remains endemic in Pakistan especially in the lower socioeconomic population where the vaccine coverage is low. The ketogenic diet has been used in a large number of diseases especially intractable epilepsies in children and also in certain neurodegenerative disorders like Parkinson's and Alzheimer's in adults. Some of the case reports from India and the USA have reported the beneficial results of ketogenic diet(KD) not only in the control of myoclonic jerks of SSPE but also in stopping and reversing the progress of the disease. There is a definite need to conduct a multi-center trial of KD to establish its effectiveness in SSPE.
At the Aga khan university, the investigators have an experienced team who have been using KD for the last 9 years for intractable epilepsy. At AKU the investigators currently have 4 patients on board for the past 4 months, with diagnosed SSPE on Low Glycemic Index Diet and showing some improvement in their seizure and motor control. Based on these reports, for a poor measles control country like Pakistan, there is a need to work further in order to establish evidence for KD therapy as a treatment for SSPE. The findings of the proposed study will serve as a hope for the improvement of quality of life of patients suffering from SSPE which is a devastating disease with almost 100% mortality .
Methodology This will be a bi-center trial. The national institute of child health (NICH) will be a partner in this study along with Aga Khan University Hospital, Karachi. This study will be a nutritional research study looking at the role of the Ketogenic diet in improving seizures and overall disability in children with SSPE. LGIT will be the form of KD used for these patients.
All families with children who have been newly diagnosed with confirmed SSPE will be approached to start the low glycemic index diet,(liberalized kind of KD). They will be counseled on the disease and the diet regimen. all possible side effects and management will be discussed. The duration of follow-up will be for one year and there will be no potential cost incurred by the patient.
Patients will also be followed up by the physiotherapist at the physiotherapy department at AKU ( @ no cost)who will be supporting their motor rehabilitation. Investigations which include blood work and echo and ultrasound and EEGs will be done at regular intervals Sampling technique A consecutive sampling of all children who are diagnosed with SSPE will be used. A total of 26 children >2-<15 years will be enrolled over a period of two years.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Ketogenic Diet | Other | This will be a prospective cohort study. All families with children who have been newly diagnosed with confirmed SSPE (based on Dyken's Criteria) will be approached to start the low glycemic index diet (liberalized kind of KD). A thorough dietary history will be taken from the parents/caregivers, and they will be counseled by a trained dietitian on the diet, all pros and cons, and the format. After the verbal agreement, formal consent will be taken from the parent/ caregiver for initiation of the diet. |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| KETOGENIC DIET | Other | This will be a prospective cohort study. All families with children who have been newly diagnosed with confirmed SSPE (based on Dyken's Criteria) will be approached to start the low glycemic index diet (liberalized kind of KD). A thorough dietary history will be taken from the parents/caregivers, and they will be counseled by a trained dietitian on the diet, all pros and cons, and the format. After the verbal agreement, formal consent will be taken from the parent/ caregiver for initiation of the diet. These patients are usually already admitted in the hospital for initial diagnosis and management. Therefore the initiation of the diet will be in the hospital during their admission if the parents/ caregivers agrees. A minimum of 2-3 days would be required to train the parents/caregivers. |
| Measure | Description | Time Frame |
|---|---|---|
| Seizure control | frequency and severity will be measured through a seizure record diary, daily number of seziures will be averaged over a month and monthly record will be evaluated as percentage increase or decrease in 3 months intervals | for a period of 6 months . |
| Measure | Description | Time Frame |
|---|---|---|
| cognitive Change , | checked by using Pedicat with 4 domains. Daily activities, Mobility and Social/Cognitive domains.The three functional skill domains are rated on a 4 point scale:
0.I don't know Responsibility:This domain has its own 5 point scale:
|
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Inclusion Criteria:• All patients with a recent diagnosis of SSPE based on Dyken's criteria. Children aged ≥2 - ≤15 years.
• Having at least one member of the family residing with the patient who can read and understand directions in Urdu and /or Sindhi.
Exclusion Criteria:
• Parents not agreeing to start the diet.
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| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Shahnaz H Ibrahim, MBBS;FCPS | Contact | +923002119347 | shahnaz.ibrahim@aku.edu | |
| Hira Farooq, NUTRITION | Contact | +923152566548 | hira.farooq@aku.edu |
| Name | Affiliation | Role |
|---|---|---|
| Shahnaz H Ibrahim, MBBS ;FCPS | AKU | Principal Investigator |
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| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 25149833 | Result | Rafique A, Amjad N, Chand P, Zaidi SS, Rana MS, Ahmed K, Ibrahim S. Subacute sclerosing panencephalitis: clinical and demographic characteristics. J Coll Physicians Surg Pak. 2014 Aug;24(8):557-60. | |
| 25232151 | Result | Ibrahim SH, Amjad N, Saleem AF, Chand P, Rafique A, Humayun KN. The upsurge of SSPE--a reflection of national measles immunization status in Pakistan. J Trop Pediatr. 2014 Dec;60(6):449-53. doi: 10.1093/tropej/fmu050. Epub 2014 Sep 16. |
| Label | URL |
|---|---|
| Jafri SK, Kumar R, Ibrahim SH. Subacute sclerosing panencephalitis - current perspectives. Pediatric Health Med Ther. 2018;9:67-71 | View source |
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| ID | Term |
|---|---|
| D013344 | Subacute Sclerosing Panencephalitis |
| D000069279 | Drug Resistant Epilepsy |
| ID | Term |
|---|---|
| D018792 | Encephalitis, Viral |
| D020805 | Central Nervous System Viral Diseases |
| D002494 | Central Nervous System Infections |
| D007239 | Infections |
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| ID | Term |
|---|---|
| D055423 | Diet, Ketogenic |
| ID | Term |
|---|---|
| D050528 | Diet, Carbohydrate-Restricted |
| D004035 | Diet Therapy |
| D044623 | Nutrition Therapy |
| D013812 | Therapeutics |
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| base line, after 6 months of study then after and one year of study. |
| behavioral Change | this will be checked by using modified Ashworth scales.MODIFIED ASHWORTH The modified Ashworth scale is a muscle tone assessment scale used to assess the resistance experienced during passive range of motion, which does not require any instrumentation and is quick to perform. scoring 0 No increase in tone 1 slight increase in tone giving a catch when slight increase in muscle tone, manifested by the limb was moved in flexion or extension. 1+ slight increase in muscle tone, manifested by a catch followed by minimal resistance throughout (ROM ) 2 more marked increase in tone but more marked increased in muscle tone through most limb easily flexed 3 considerable increase in tone, passive movement difficult 4 limb rigid in flexion or extension | base line, after 6 months of study then after and one year of study |
| motor developmental Change | this will be checked by , Modified Rankin and GMFM Modified Rankin Scale Score Description 0 No symptoms at all
The scoring system of the GMFM is a four-point scale that consists of 66 items divided into five dimensions of gross motor function:(a) lying and rolling, (b) sitting, (c) crawling and kneeling, (d) standing, and (e) walking, running and jumping. | base line, after 6 months of study then after and one year of study |
| D000069544 | Infectious Encephalitis |
| D014777 | Virus Diseases |
| D008457 | Measles |
| D018185 | Morbillivirus Infections |
| D018184 | Paramyxoviridae Infections |
| D018701 | Mononegavirales Infections |
| D012327 | RNA Virus Infections |
| D012897 | Slow Virus Diseases |
| D004660 | Encephalitis |
| D001927 | Brain Diseases |
| D002493 | Central Nervous System Diseases |
| D009422 | Nervous System Diseases |
| D000090862 | Neuroinflammatory Diseases |
| D004827 | Epilepsy |
| D004032 |
| Diet |
| D009747 | Nutritional Physiological Phenomena |
| D000066888 | Diet, Food, and Nutrition |
| D010829 | Physiological Phenomena |