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KMID : 1140920180420020363
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Annals of Rehabilitation Medicine
2018 Volume.42 No. 2 p.363 ~ p.367
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Botulinum Toxin-A Injection in theTreatment of Spasticity in a Infantile-OnsetNeurodegeneration With Brain IronAccumulation: A Case Report
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Do Hwan-Kwon
Jo Geun-Yeol
Kwon Jun-Koo
Kim Woo-Jin
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Abstract
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Pantothenate kinase-associated neurodegeneration (PKAN) is a neurodegenerative disorder characterized byiron accumulation in the globus pallidus (GP) of the brain (neurodegeneration with brain iron accumulation[NBIA]), which is characterized by dystonia and spasticity resulting in postural difficulties. A 33-month-old boywas admitted with a pronounced gait disturbance. Marked hypertonicity in the patient’s both calf muscles wasnoted, resulting in waddling with repeated slip-falls. NBIA was suspected by high T2 intensity in the GP on brainMRI, then it was confirmed by detecting PANK2 mutation. Botulinum toxin-A injection was administered toboth calf muscles. After 2 weeks, a decrease in spasticity and an increase in range of motion were observed, andconsequently, an increase in the patient’s gait stability with both heels touching the ground, enabling him to walkstraight independently. A definitive treatment for NBIA has not been established, and a symptomatic therapyis currently the mainstay of treatment in this case. This is the first case report of botulinum toxin injection fortreatment of gait disturbance caused by spasticity in an infantile-onset PKAN.
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KEYWORD
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Neurodegeneration with brain iron accumulation (NBIA), Pantothenate kinase-associated neurodegeneration (PKAN), PANK2
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