Maple Syrup Urine Disease Induced Grand Mal Seizures: A Case Report (Case Report)

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Satheesh Gautam; Johnson Suja; Guhan Balraj; Ahammed Niyas

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Journal Paper

Paper Information

Journal: Journal of Pediatric Perspectives Year:2018 | Volume:6 | Issue:7 (55) Page(s): 7999-8002

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Information Journal Paper

Title

Maple Syrup Urine Disease Induced Grand Mal Seizures: A Case Report (Case Report)

Author(s)

Satheesh Gautam | Johnson Suja | Guhan Balraj | Ahammed Niyas | Issue Writer Certificate

Keywords

GTCS

Genetic

MSUD

Seizures

Quadriplegia

Abstract

Background Maple Syrup Urine Disease (MSUD) is a rare autosomal recessive metabolic error, characterized by Branched Chain α-Keto-acid Dehydrogenase Complex (BCKDC) deficiency. Mutations in 3 genes can lead to abnormal metabolism and accumulation of leucine, isoleucine, valine and corresponding keto-acids. MSUD affects 1 in 185, 000 infants globally. Seizure is a common presentation among neonates. However, in intermediate MSUD, Seizures have a delayed and insidious onset, along with developmental Case Report We report a case of grand mal Seizures in a patient with intermediate MSUD, presenting with multiple episodes of seizure, dystonia, spastic Quadriplegia, involuntary micturition and oculogyric crisis. Seizures were managed successfully with intravenous lorazepam and other supportive measures. The patient was advised to strictly adhere to branched chain amino acid restricted diet. Conclusion This case report emphasizes on the importance of medication adherence and dietary restrictions to prevent permanent psychomotor damage or death.

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APA: Copy

Satheesh, Gautam, Johnson, Suja, Guhan, Balraj, & Ahammed, Niyas. (2018). Maple Syrup Urine Disease Induced Grand Mal Seizures: A Case Report (Case Report). INTERNATIONAL JOURNAL OF PEDIATRICS, 6(7 (55)), 7999-8002. SID. https://sid.ir/paper/337542/en

Vancouver: Copy

Satheesh Gautam, Johnson Suja, Guhan Balraj, Ahammed Niyas. Maple Syrup Urine Disease Induced Grand Mal Seizures: A Case Report (Case Report). INTERNATIONAL JOURNAL OF PEDIATRICS[Internet]. 2018;6(7 (55)):7999-8002. Available from: https://sid.ir/paper/337542/en

IEEE: Copy

Gautam Satheesh, Suja Johnson, Balraj Guhan, and Niyas Ahammed, “Maple Syrup Urine Disease Induced Grand Mal Seizures: A Case Report (Case Report),” INTERNATIONAL JOURNAL OF PEDIATRICS, vol. 6, no. 7 (55), pp. 7999–8002, 2018, [Online]. Available: https://sid.ir/paper/337542/en

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