Available online 10 April 2024, 101123

Author links open overlay panel, ABSTRACT

Acute neuromuscular disorders occasionally occur in the Pediatric Neurologic Intensive Care Unit. Many of these are primary disorders of the motor unit that may present acutely or exacerbate during an intercurrent illness. Additionally, acute neuromuscular disorders may develop during an acute systemic illness requiring intensive care management that predispose the child to another set of acute motor unit disorders. This chapter discusses acute neuromuscular crises in the infant, toddler, and adolescent, as well as neuromuscular disorders resulting from critical illness.

Section snippetsINTRODUCTION

Acute neuromuscular disorders occasionally occur in the pediatric intensive care unit. Many of these are primary motor unit disorders that present acutely and require admission to a critical care unit. We divide these disorders into two age groups, infants and children: some disorders are unique to infants and toddlers and the presenting manifestations may vary by age.1,2 Although this creates somewhat artificial categories, the format is practical for evaluating childhood acute weakness. A

ACUTE NEUROMUSCULAR CRISES IN THE INFANT

Newborns may present with floppy infant syndrome, or infants acutely with a flaccid paralysis following a healthy first few months of life.5 Some newborns presenting primarily with an acute respiratory compromise are concomitantly observed to be hypotonic, sometimes to the degree of being identified as a “floppy baby.” A variety of congenital or developmental lesions at any level of the peripheral motor unit may lead to this clinical picture (Table 1). Specific neuromuscular diagnoses that must

ACUTE NEUROMUSCULAR CRISES IN THE TODDLER AND THE OLDER CHILD

The presentation of acute weakness the older child has a broader differential diagnosis including both acquired and genetic pathologies that localized to various levels of the neuromuscular system (Table 2).

CRITICAL ILLNESS NEUROMUSCULAR DISORDERS

The acute weakness or paralysis which may occasionally occur in children with critical illness may be related to peripheral nerve, endplate, or muscle disorders, collectively, referred to as critical illness associated weakness (CIAW). Children with overwhelming sepsis or status asthmaticus may develop CIAW that may mimic a hospital-acquired GBS presenting with a failure to wean from respirator.96, 97, 98 Bolton was one of the first to emphasize the need to consider these various syndromes in

CONCLUSIONS

The presentation of acute flaccid paralysis in neonates, infants and older children has a spectrum of etiologies that are best thought through within the anatomic framework of the motor unit. By systematically thinking through pathologies that localize to the anterior horn cell, nerve, neuromuscular junction and muscle, the clinician can generate an appropriate differential diagnosis based on the age and clinical presentation of the patient. Electrodiagnostic studies can greatly aid supporting

CRediT authorship contribution statement

Leslie H. Hayes: Conceptualization, Data curation, Writing – original draft. Basil T. Darras: Conceptualization, Data curation, Writing – review & editing.

Declaration of competing interest

The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

References (138)GG Shapiro et al.Poliomyelitis-like illness after acute asthma

The Journal of pediatrics

(1979)

K Nihei et al.Poliomyelitis-like syndrome following asthmatic attack (Hopkins syndrome)

Pediatr Neurol

(1987)

SC Yeung et al.Flaccid paralysis of the limbs after an asthmatic attack

Pediatr Neurol

(2010)

JJ Sejvar et al.Guillain-Barré syndrome and Fisher syndrome: case definitions and guidelines for collection, analysis, and presentation of immunization safety data

Vaccine

(2011)

MC Durand et al.Clinical and electrophysiological predictors of respiratory failure in Guillain-Barré syndrome: a prospective study

The Lancet Neurology

(2006)

MM Ryan et al.Paediatric mononeuritis multiplex: a report of three cases and review of the literature

Neuromuscul Disord

(2003)

P Mullaney et al.The natural history and ophthalmic involvement in childhood myasthenia gravis at the hospital for sick children

Ophthalmology

(2000)

RD Sheth et al.Critical illness neuromuscular disease in children manifested as ventilatory dependence

The Journal of pediatrics

(1995)

HR. JonesEvaluation of the floppy infantWS David et al.Electromyography and biopsy correlation with suggested protocol for evaluation of the floppy infant

Muscle Nerve

(1994)

CS Ryan et al.Nerve conduction normal values for electrodiagnosis in pediatric patients

Muscle Nerve

(2019)

HRJ Jones et al.An approach to pediatric electromyographyMC Keinath et al.Spinal Muscular Atrophy: Mutations, Testing, and Clinical Relevance

Appl Clin Genet

(2021)

S Pekuz et al.Spinal muscular atrophy with respiratory distress type 1 (SMARD1): a rare cause of hypotonia, diaphragmatic weakness, and respiratory failure in infants

Turk J Pediatr

(2022)

MGL Perego et al.Current understanding of and emerging treatment options for spinal muscular atrophy with respiratory distress type 1 (SMARD1)

Cell Mol Life Sci

(2020)

PB Kang et al.Diagnostic value of electromyography and muscle biopsy in arthrogryposis multiplex congenita

Ann Neurol

(2003)

AH Jackson et al.Congenital Guillain-Barré syndrome

J Child Neurol

(1996)

A Rolfs et al.Guillain-Barré syndrome in pregnancy: reflections on immunopathogenesis

Acta Neurol Scand

(1994)

HR. JonesChildhood Guillain-Barré syndrome: clinical presentation, diagnosis, and therapy

J Child Neurol

(1996)

RC Gilmartin et al.Guillain-Barré syndrome with hydrocephalus in early infancy

Archives of neurology

(1977)

JF Pasternak et al.An infant with chronic, relapsing polyneuropathy responsive to steroids

Dev Med Child Neurol

(1982)

PJ Grattan-Smith et al.Clinical and neurophysiological features of tick paralysis

Brain

(1997)

O. PapazianTransient neonatal myasthenia gravis

J Child Neurol

(1992)

AK Lefvert et al.Newborn infants to myasthenic mothers: a clinical study and an investigation of acetylcholine receptor antibodies in 17 children

Neurology

(1983)

P O'Carroll et al.Transient neonatal myasthenia gravis in a baby born to a mother with new-onset anti-MuSK-mediated myasthenia gravis

J Clin Neuromuscul Dis

(2009)

NE Gilhus et al.Maternal myasthenia gravis represents a risk for the child through autoantibody transfer, immunosuppressive therapy and genetic influence

Eur J Neurol

(2018)

PJ. LipsitzThe clinical and biochemical effects of excess magnesium in the newborn

Pediatrics

(1971)

MM Sokal et al.Neonatal hypermagnesemia and the meconium-plug syndrome

The New England journal of medicine

(1972)

AK Rao et al.Clinical Guidelines for Diagnosis and Treatment of Botulism, 2021

MMWR Recomm Rep

(2021)

SS Arnon et al.Human botulism immune globulin for the treatment of infant botulism

The New England journal of medicine

(2006)

J Pickett et al.Syndrome of botulism in infancy: clinical and electrophysiologic study

The New England journal of medicine

(1976)

SA Clay et al.Acute infantile motor unit disorder. Infantile botulism?

Archives of neurology

(1977)

RE Hoffman et al.Type F infant botulism

Am J Dis Child

(1982)

RH Schwartz et al.Infant botulism: exacerbation by aminoglycosides

Am J Dis Child

(1982)

JA Thompson et al.Infant botulism: clinical spectrum and epidemiology

Pediatrics

(1980)

ML May et al.Infant botulism in Australia: availability of human botulinum antitoxin for treatment

Med J Aust

(2010)

C Chalk et al.Medical treatment for botulism

Cochrane Database Syst Rev

(2011)

KA Grant et al.Report of two unlinked cases of infant botulism in the UK in October 2007

J Med Microbiol

(2009)

DR Cornblath et al.Clinical electrophysiology of infantile botulism

Muscle Nerve

(1983)

RD Sheth et al.Infantile botulism: pitfalls in electrodiagnosis

J Child Neurol

(1999)

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