Botulinum toxin injection to improve functional independence and to alleviate parenting stress in a child with advanced pantothenate kinase-associated neurodegeneration

Cho I. Lin, Kuan-Lin Chen, Da-Shen Kuan, Sheng Han Lin, Wei Pin Lin, Yu-Ching Lin

Research output: Contribution to journalReview article

1 Citation (Scopus)

Abstract

Rationale: Pantothenate kinase-associated neurodegeneration (PKAN) is a rare autosomal recessive disease. Progressive motor symptoms such as dystonia and spasticity begin in childhood and relentlessly become incapacitating later in life. Treatments including anticholinergics and iron chelation are usually ineffective. Botulinum toxin type A (BoNT-A) is effective for adult patients with dystonia or spasticity. Patient concerns: We reported a 10-year-old female patient with advanced PKAN, manifesting as generalized dystonia and spasticity. Diagnosis: The patient was diagnosed with PKAN by a pediatric neurologist. Interventions: The patient received BoNT-A injection. Outcomes: The effect was obvious at four weeks after the injection, with an improvement of 25% in Barry-Albright Dystonia Scale and 4% in Functional Independence Measure for Children score. Furthermore, there was a 3.8% reduction in Parenting Stress Index Short Form score and 8.3% improvement in Pain and Impact of Disability domain in the score of Cerebral Palsy Quality of Life for Children. Lessons: BoNT-A injection was effective to improve functional independence and to alleviate stress of caregivers in the patient with advanced PKAN.

Original languageEnglish
Article numbere10709
JournalMedicine (United States)
Volume97
Issue number20
DOIs
Publication statusPublished - 2018 May 1

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Pantothenate Kinase-Associated Neurodegeneration
Botulinum Toxins
Parenting
Dystonia
Type A Botulinum Toxins
Injections
Cholinergic Antagonists
Cerebral Palsy
Caregivers
Iron
Quality of Life
Pediatrics
Pain

All Science Journal Classification (ASJC) codes

  • Medicine(all)

Cite this

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title = "Botulinum toxin injection to improve functional independence and to alleviate parenting stress in a child with advanced pantothenate kinase-associated neurodegeneration",
abstract = "Rationale: Pantothenate kinase-associated neurodegeneration (PKAN) is a rare autosomal recessive disease. Progressive motor symptoms such as dystonia and spasticity begin in childhood and relentlessly become incapacitating later in life. Treatments including anticholinergics and iron chelation are usually ineffective. Botulinum toxin type A (BoNT-A) is effective for adult patients with dystonia or spasticity. Patient concerns: We reported a 10-year-old female patient with advanced PKAN, manifesting as generalized dystonia and spasticity. Diagnosis: The patient was diagnosed with PKAN by a pediatric neurologist. Interventions: The patient received BoNT-A injection. Outcomes: The effect was obvious at four weeks after the injection, with an improvement of 25{\%} in Barry-Albright Dystonia Scale and 4{\%} in Functional Independence Measure for Children score. Furthermore, there was a 3.8{\%} reduction in Parenting Stress Index Short Form score and 8.3{\%} improvement in Pain and Impact of Disability domain in the score of Cerebral Palsy Quality of Life for Children. Lessons: BoNT-A injection was effective to improve functional independence and to alleviate stress of caregivers in the patient with advanced PKAN.",
author = "Lin, {Cho I.} and Kuan-Lin Chen and Da-Shen Kuan and Lin, {Sheng Han} and Lin, {Wei Pin} and Yu-Ching Lin",
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T1 - Botulinum toxin injection to improve functional independence and to alleviate parenting stress in a child with advanced pantothenate kinase-associated neurodegeneration

AU - Lin, Cho I.

AU - Chen, Kuan-Lin

AU - Kuan, Da-Shen

AU - Lin, Sheng Han

AU - Lin, Wei Pin

AU - Lin, Yu-Ching

PY - 2018/5/1

Y1 - 2018/5/1

N2 - Rationale: Pantothenate kinase-associated neurodegeneration (PKAN) is a rare autosomal recessive disease. Progressive motor symptoms such as dystonia and spasticity begin in childhood and relentlessly become incapacitating later in life. Treatments including anticholinergics and iron chelation are usually ineffective. Botulinum toxin type A (BoNT-A) is effective for adult patients with dystonia or spasticity. Patient concerns: We reported a 10-year-old female patient with advanced PKAN, manifesting as generalized dystonia and spasticity. Diagnosis: The patient was diagnosed with PKAN by a pediatric neurologist. Interventions: The patient received BoNT-A injection. Outcomes: The effect was obvious at four weeks after the injection, with an improvement of 25% in Barry-Albright Dystonia Scale and 4% in Functional Independence Measure for Children score. Furthermore, there was a 3.8% reduction in Parenting Stress Index Short Form score and 8.3% improvement in Pain and Impact of Disability domain in the score of Cerebral Palsy Quality of Life for Children. Lessons: BoNT-A injection was effective to improve functional independence and to alleviate stress of caregivers in the patient with advanced PKAN.

AB - Rationale: Pantothenate kinase-associated neurodegeneration (PKAN) is a rare autosomal recessive disease. Progressive motor symptoms such as dystonia and spasticity begin in childhood and relentlessly become incapacitating later in life. Treatments including anticholinergics and iron chelation are usually ineffective. Botulinum toxin type A (BoNT-A) is effective for adult patients with dystonia or spasticity. Patient concerns: We reported a 10-year-old female patient with advanced PKAN, manifesting as generalized dystonia and spasticity. Diagnosis: The patient was diagnosed with PKAN by a pediatric neurologist. Interventions: The patient received BoNT-A injection. Outcomes: The effect was obvious at four weeks after the injection, with an improvement of 25% in Barry-Albright Dystonia Scale and 4% in Functional Independence Measure for Children score. Furthermore, there was a 3.8% reduction in Parenting Stress Index Short Form score and 8.3% improvement in Pain and Impact of Disability domain in the score of Cerebral Palsy Quality of Life for Children. Lessons: BoNT-A injection was effective to improve functional independence and to alleviate stress of caregivers in the patient with advanced PKAN.

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