Original Article Acute Neuromuscular Disorders in the Pediatric Intensive Care Unit Dana B. Harrar, MD, PhD 1,2 , Basil T. Darras, MD 1 , and Partha S. Ghosh, MD 1 Abstract Background: The neuromuscular disorders encountered in the pediatric intensive care unit (PICU) encompass a broad spectrum of pathologies. These include acute disorders (eg, Guillain-Barre syndrome), acute-on-chronic disorders (eg, myas- thenia gravis), progressive disorders (eg, muscular dystrophy), and disorders that develop in the PICU (eg, critical illness myo- pathy/polyneuropathy). Familiarity with the presenting features of these disorders is of paramount importance in facilitating timely diagnosis. Methods: We conducted a retrospective review of the medical records of patients admitted to the PICU or Inter- mediate Care Program (ICP) at a single tertiary children’s hospital from 2006 to 2017 with an acute or acute-on-chronic neu- romuscular disorder. We did not include patients with a known progressive neuromuscular disorder or critical illness myopathy/ polyneuropathy. Results: Twenty-four patients were admitted to the PICU/ICP with acute or acute-on-chronic neuromuscular disorders. Diagnosis and indication for ICU/ICP admission were Guillain-Barre syndrome (n ¼ 6; respiratory failure: 3, respiratory monitoring: 2, autonomic instability: 1), myasthenia gravis (n ¼ 5; airway clearance: 3, respiratory failure: 2), acute flaccid myelitis (n ¼ 3; respiratory failure: 2, respiratory monitoring: 1), periodic paralysis (n ¼ 3; intravenous potassium replacement), rhab- domyolysis (n ¼ 3; monitoring for electrolyte derangements), infant botulism (n ¼ 2; respiratory failure), chronic demyelinating polyneuropathy (n ¼ 1; respiratory failure), and congenital myasthenic syndrome (n ¼ 1; apnea). No patients were admitted to the PICU/ICP with a diagnosis of tick paralysis, acute intermittent porphyria, or inflammatory myopathy. Conclusions: Although acute and acute-on-chronic neuromuscular disorders are encountered relatively rarely in the PICU, familiarity with the presenting features of these disorders is important in facilitating timely diagnosis. This, in turn, enables the institution of effective management strategies, thereby avoiding complications associated with diagnostic delays. Keywords neuromuscular disorder, acute flaccid myelitis, inflammatory demyelinating polyneuropathy, myasthenia, botulism, periodic paralysis, rhabdomyolysis Received May 4, 2019. Received revised July 8, 2019. Accepted for publication July 30, 2019. The neuromuscular disorders encountered in the pediatric intensive care unit (PICU) encompass a broad spectrum of pathologies. 1 These include acute disorders, often seen in pre- viously healthy patients, such as Guillain-Barre syndrome and infant botulism, acute-on-chronic disorders such as myasthenia gravis and periodic paralysis, progressive disorders such as the inherited myopathies and muscular dystrophies, and disorders that develop in the intensive care unit in the form of critical illness myopathies and polyneuropathies. The differential diag- nosis of neuromuscular problems in the critically-ill neonate and child was outlined in 2004 by Darras and Jones based on 24 years of experience in the electromyography laboratory at a tertiary children’s hospital. 2 Yates et al 3 has described the out- come of patients with progressive neuromuscular disorders admitted to a pediatric intensive care unit, including patients with spinal muscular atrophy, muscular dystrophies, noninflammatory myopathies, and congenital and hereditary neuropathies, whereas Banwell et al 4 reported the incidence and characteristics of weakness developing in critically-ill patients in the pediatric intensive care unit. To our knowledge, the frequency with which pediatric intensivists and intensive care neurologists are likely to encounter acute and acute-on- chronic neuromuscular disorders has not been reported. Here, 1 Department of Neurology, Boston Children’s Hospital, Boston, MA, USA 2 Division of Neurology, Children’s National Medical Center, Washington, DC, USA Corresponding Author: Dana B. Harrar, MD, PhD, Division of Neurology, Children’s National Medical Center, Washington, DC 20010, USA. Email: [email protected] Journal of Child Neurology 2020, Vol. 35(1) 17-24 ª The Author(s) 2019 Article reuse guidelines: sagepub.com/journals-permissions DOI: 10.1177/0883073819871437 journals.sagepub.com/home/jcn
Welcome message from author
This document is posted to help you gain knowledge. Please leave a comment to let me know what you think about it! Share it to your friends and learn new things together.
Transcript
Acute Neuromuscular Disorders in the Pediatric Intensive Care UnitAcute Neuromuscular Disorders in the Pediatric Intensive Care Unit
Dana B. Harrar, MD, PhD1,2 , Basil T. Darras, MD1, and Partha S. Ghosh, MD1
Abstract
Background: The neuromuscular disorders encountered in the pediatric intensive care unit (PICU) encompass a broad spectrum of pathologies. These include acute disorders (eg, Guillain-Barre syndrome), acute-on-chronic disorders (eg, myas- thenia gravis), progressive disorders (eg, muscular dystrophy), and disorders that develop in the PICU (eg, critical illness myo- pathy/polyneuropathy). Familiarity with the presenting features of these disorders is of paramount importance in facilitating timely diagnosis. Methods: We conducted a retrospective review of the medical records of patients admitted to the PICU or Inter- mediate Care Program (ICP) at a single tertiary children’s hospital from 2006 to 2017 with an acute or acute-on-chronic neu- romuscular disorder. We did not include patients with a known progressive neuromuscular disorder or critical illness myopathy/ polyneuropathy. Results: Twenty-four patients were admitted to the PICU/ICP with acute or acute-on-chronic neuromuscular disorders. Diagnosis and indication for ICU/ICP admission were Guillain-Barre syndrome (n¼ 6; respiratory failure: 3, respiratory monitoring: 2, autonomic instability: 1), myasthenia gravis (n ¼ 5; airway clearance: 3, respiratory failure: 2), acute flaccid myelitis (n ¼ 3; respiratory failure: 2, respiratory monitoring: 1), periodic paralysis (n ¼ 3; intravenous potassium replacement), rhab- domyolysis (n ¼ 3; monitoring for electrolyte derangements), infant botulism (n ¼ 2; respiratory failure), chronic demyelinating polyneuropathy (n ¼ 1; respiratory failure), and congenital myasthenic syndrome (n ¼ 1; apnea). No patients were admitted to the PICU/ICP with a diagnosis of tick paralysis, acute intermittent porphyria, or inflammatory myopathy. Conclusions: Although acute and acute-on-chronic neuromuscular disorders are encountered relatively rarely in the PICU, familiarity with the presenting features of these disorders is important in facilitating timely diagnosis. This, in turn, enables the institution of effective management strategies, thereby avoiding complications associated with diagnostic delays.
Keywords neuromuscular disorder, acute flaccid myelitis, inflammatory demyelinating polyneuropathy, myasthenia, botulism, periodic paralysis, rhabdomyolysis
Received May 4, 2019. Received revised July 8, 2019. Accepted for publication July 30, 2019.
The neuromuscular disorders encountered in the pediatric
intensive care unit (PICU) encompass a broad spectrum of
pathologies.1 These include acute disorders, often seen in pre-
viously healthy patients, such as Guillain-Barre syndrome and
infant botulism, acute-on-chronic disorders such as myasthenia
gravis and periodic paralysis, progressive disorders such as the
inherited myopathies and muscular dystrophies, and disorders
that develop in the intensive care unit in the form of critical
illness myopathies and polyneuropathies. The differential diag-
nosis of neuromuscular problems in the critically-ill neonate
and child was outlined in 2004 by Darras and Jones based on 24
years of experience in the electromyography laboratory at a
tertiary children’s hospital.2 Yates et al3 has described the out-
come of patients with progressive neuromuscular disorders
admitted to a pediatric intensive care unit, including patients
with spinal muscular atrophy, muscular dystrophies,
noninflammatory myopathies, and congenital and hereditary
neuropathies, whereas Banwell et al4 reported the incidence
and characteristics of weakness developing in critically-ill
patients in the pediatric intensive care unit. To our knowledge,
the frequency with which pediatric intensivists and intensive
care neurologists are likely to encounter acute and acute-on-
chronic neuromuscular disorders has not been reported. Here,
1 Department of Neurology, Boston Children’s Hospital, Boston, MA, USA 2 Division of Neurology, Children’s National Medical Center, Washington, DC,
USA
Corresponding Author:
Dana B. Harrar, MD, PhD, Division of Neurology, Children’s National Medical
Center, Washington, DC 20010, USA.
Email: [email protected]
acute and acute-on-chronic neuromuscular disorders in the
Pediatric Intensive Care Unit and Intermediate Care Program
at a tertiary children’s hospital.
Material and Methods
We conducted a retrospective review of the medical records of
patients admitted to the PICU and Intermediate Care Program (ICP)
at a tertiary children’s hospital between August 2006 and September
2017 for one of the following acute or acute-on-chronic neuromuscu-
lar disorders: acute flaccid myelitis, acute intermittent porphyria,
chronic inflammatory demyelinating polyneuropathy, congenital
myasthenic syndrome, Guillain-Barre syndrome, infant botulism,
inflammatory myopathy, myasthenia gravis, periodic paralysis, rhab-
domyolysis, and tick paralysis. The Intermediate Care Program pro-
vides a level of care intermediate between that of the ICU and the
general neurology ward. Patients were identified by searching the
electronic medical record using a centralized repository of clinical
data based on ICD-9 and -10 codes. For patients admitted to the ICU
or ICP multiple times during this 11-year period, we reviewed the first
admission to the ICU/ICP in detail and recorded the number of sub-
sequent admissions to the hospital during the first year after the initial
ICU/ICP admission but did not review subsequent admissions in
detail. We reviewed the patients’ charts for demographic information,
clinical characteristics, diagnostic investigations, treatment, and clin-
ical course. We did not include patients with known progressive dis-
orders, for example, spinal muscular atrophy, muscular dystrophy, and
noninflammatory myopathies, and disorders that develop in the inten-
sive care unit as a result of critical illness, for example, critical illness
myopathy and critical illness polyneuropathy, given that these cohorts
have been described previously.3,4 Study data were collected and
managed using Research Electronic Data Capture (REDCap) tools
hosted at Boston Children’s Hospital.5 We used descriptive statistics
as needed. All procedures performed in studies involving human
participants were in accordance with the ethical standards of the
Institutional Research Ethics Board of Boston Children’s Hospital
(IRB-P00024179). Consent was not required.
Results
Twenty-four patients were admitted to the Pediatric Intensive
Care Unit or Intermediate Care Program with an acute or acute-
on-chronic neuromuscular disorder between August 2006 and
September 2017. Approximately 3100 patients are admitted to
our pediatric intensive care unit each year, indicating that
admissions for acute and acute-on-chronic neuromuscular dis-
orders account for less than 0.1% of PICU admissions per year.
Diagnoses were as follows: Guillain-Barre syndrome (n ¼ 6),
myasthenia gravis (n ¼ 5), acute flaccid myelitis (n ¼ 3),
periodic paralysis (n ¼ 3), rhabdomyolysis (n ¼ 3), infant
botulism (n ¼ 2), chronic inflammatory demyelinating poly-
neuropathy (n¼ 1), and congenital myasthenic syndrome (n¼ 1).
No patients were admitted to the PICU or ICP within the 11-year
study period with a diagnosis of acute intermittent porphyria,
inflammatory myopathy, or tick paralysis. Nineteen (79%)
patients presented with a previously undiagnosed neuromuscular
disorder, 14 (74%) of whom were diagnosed prior to discharge.
Almost half of the patients in our cohort required ICU-level care
for invasive or noninvasive respiratory support, and respiratory
indications were the most common reason for admission to the
ICU/ICP (n ¼ 17, 71%). A majority of the neuromuscular
disorders encountered in our ICU over the past 11 years have
treatment options that, when initiated promptly, target the
underlying pathophysiologic process; in keeping with this, 17
(71%) patients were treated with potentially disease-modifying
therapeutics. One-third (n ¼ 8) of the patients in our cohort
experienced complications during their ICU admission, all of
whom were admitted for 9 days or more, and no patient died
as a complication of their neuromuscular disorder. We divide
these disorders below according to their localization in the
lower motor unit pathway.
Acute Flaccid Myelitis
Three patients had acute flaccid myelitis (Tables 1-3). All 3
patients were diagnosed in either 2014 or 2016, years during
which there was a national increase in reports of acute flaccid
myelitis. All of them had axial and limb weakness and
depressed deep tendon reflexes in the affected limbs. Cere-
brospinal fluid analysis demonstrated pleocytosis with a lym-
phocytic predominance in 3/3 patients and an elevated protein
in 2/3 patients; testing of the serum and cerebrospinal fluid for
infectious agents, including enterovirus, was negative. Neuroi-
maging revealed hyperintensity of the central gray matter of the
spinal cord in 3/3 patients, hyperintensity of the dorsal brain-
stem in 2/3 patients, and abnormal enhancement of the cauda
equina in 1/3 patients. Two patients required respiratory sup-
port, including one who required a tracheostomy. All patients
received intravenous immunoglobulin infusions. Additional
treatments included plasmapheresis (n ¼ 2), corticosteroids
(n ¼ 2), and fluoxetine (n ¼ 1), none of which are currently
favored treatment approaches.6 Two patients had a prolonged
(2 weeks) ICU/ICP admission, one complicated by
pneumonia and the other by tracheitis. At the time of dis-
charge, 2 patients were nonambulatory and 1 was ambulatory
with limitations. All 3 patients were discharged to a rehabi-
litation hospital.
Guillain-Barre Syndrome
them had a progressive ascending paralysis of the limbs. Cere-
brospinal fluid analysis revealed albuminocytologic dissocia-
tion in 2/6 patients at 5 and 14 days after symptom onset.
Cerebrospinal fluid analysis in the remaining 4 patients
revealed a normal protein level; in all 4 patients, cerebrospinal
fluid analysis was carried out within the first week after symp-
tom onset. Spine MRI revealed abnormal enhancement of the
cauda equina in 2/5 patients. Electromyography revealed
demyelinating polyneuropathy (n ¼ 1), motor axonal
18 Journal of Child Neurology 35(1)
neuropathy (n ¼ 1), or a mixed axonal and demyelinating
polyneuropathy (n ¼ 2). Three patients required respiratory
support, including 2 who required tracheostomy. All 6 patients
received intravenous immunoglobulin; additional treatments
included plasmapheresis in 3 patients and corticosteroids in
1 patient for concomitant systemic lupus erythematosus (SLE).
These patients spent a median of 24 days in the hospital with a
median of 11 days in the ICU/ICP. Complications during
admission included: pneumonia (n ¼ 3), Clostridium difficile
colitis (n ¼ 2), pneumothorax (n ¼ 1), pressure ulcer (n ¼ 1),
and UTI (n ¼ 1). Five patients were ambulatory at baseline; at
the time of discharge, 3 were no longer ambulatory and 2 were
ambulatory with limitations. Four patients were discharged to a
rehabilitation hospital, while the remaining 2 were discharged
home. The patient with acute motor axonal neuropathy was
readmitted to the hospital 8 times in the first year after his
initial ICU/ICP admission for complications related to SLE;
6 of these admissions required ICU/ICP-level care. None of
the remaining patients were re-admitted to the hospital in the
first year after admission for Guillain-Barre syndrome.
Chronic Inflammatory Demyelinating Polyneuropathy
neuropathy (CIDP) (Tables 1-3). She was admitted to the ICU
due to worsening axial and limb weakness. In addition, she
developed respiratory and bulbar dysfunction and required
intubation for almost 2 weeks. Cerebrospinal fluid analysis
revealed mild pleocytosis and a very high protein level. Spine
MRI showed diffuse thickening and enhancement of the
Table 1. Presenting Symptoms, Treatment, and Clinical Course.
Patient Age
Respiratory support Treatment
ICU days Complications
1 1/M AFM RD B; N; UE/LE; areflexia Tracheostomy Steroids, IVIG, fluoxetine
57 Tracheitis
2 4/M AFM RD B; N; UE/LE; areflexia Intubation Steroids, IVIG, PLEX 14 PNA 3 5/M AFM RM N; UE/LE; areflexia None Steroids, IVIG, PLEX 1 4 6/M GBS Autonomic EOM; Fa; B; UE/LE;
areflexia Tracheostomy Steroids, IVIG, PLEX 46 PNA, pressure ulcer,
C diff 5 16/F GBS RM Ptosis; UE/LE; areflexia None IVIG 3 6 17/M GBS RF Fa; B; UE/LE; S; areflexia BiPAP IVIG, PLEX 22 PNA, PTX, UTI 7 16/M GBS RF P; ptosis; B; UE/LE; S;
areflexia Tracheostomy IVIG, PLEX 28
8 6/F GBS RM B; N; UE/LE; areflexia None IVIG 2 9 18/M GBS RF UE/LE Tracheostomy* IVIG 11 PNA, SIADH, C diff 10 9/F CIDP RF Ptosis; EOM; B; UE/LE;
areflexia Intubation Steroids, IVIG, PLEX 15
11 13/F MG RD EOM; Fa; B; UE/LE Intubation Steroids, IVIG, Pyrido
27 PNA, UTI
12 14/F MG AC Ptosis; EOM; Fa; B; N; UE/LE
None PLEX, Pyrido 2
13 18/F MG AC Ptosis; EOM; B; UE/LE None Steroids, PLEX, Pyrido
2
14 11/F MG RF Ptosis; EOM; B; UE/LE Intubation PLEX, Pyrido 9 PNA, UTI 15 16/F MG AC Ptosis None Pyrido 1 16 0/M CMS RD P; UE/LE Tracheostomy* Supportive care 7 17 0/M IB RF B; N; UE/LE Intubation Botulism Ig 9 PNA 18 0/M IB RD B; UE/LE Intubation Botulism Ig 6 19 15/M HypoPP K repletion UE/LE None K repletion 2 20 16/M HypoPP K repletion None None K repletion 1 21 14/M HypoPP K repletion UE/LE None K repletion 1 22 2/M Rhabdo Electrolyte Gait abnormality None Supportive care 1 23 1/M Rhabdo Electrolyte UE/LE None Steroids, HCO3
fluids 5
Abbreviations: AC, airway clearance; AFM, acute flaccid myelitis; B, bulbar weakness; BiPAP, bilevel positive airway pressure; C diff, Clostridium difficile infection; CIDP, chronic inflammatory demyelinating polyneuropathy; CMS, congenital myasthenic syndrome; EOM, extraocular muscle weakness; F, female; Fa, facial weakness; GBS, Guillain-Barre syndrome; HCO3, bicarbonate; HypoPP, hypokalemic periodic paralysis; IB, infant botulism; Ig, immunoglobulin; IVIG, intravenous immunoglobulin; K, potassium; LE, lower extremity weakness; M, male; MG, myasthenia gravis; N, neck weakness; P, pupillary abnormalities; PLEX, plasmapheresis; PNA, pneumonia; PTX, pneumothorax; Pyrido, pyridostigmine; RD, respiratory distress; RF, respiratory failure; rhabdo, rhabdomyolysis; RM, respiratory monitoring; S, sensory abnormalities; SIADH, syndrome of inappropriate diuretic hormone secretion; UE, upper extremity weakness; UTI, urinary tract infection. *tracheostomy at baseline.
Harrar et al 19
linating and axonal features, and sural nerve biopsy showed
mild inflammation, focal demyelination, and the presence of
onion bulbs. An extensive infectious and paraneoplastic
work-up was negative. She was treated with intravenous
immunoglobulin, plasmapheresis, and intravenous corticos-
teroids and was then maintained on intravenous immunoglo-
bulin infusions and oral corticosteroids as an outpatient. She
spent 2 weeks in the ICU. She was ambulatory at baseline but
was nonambulatory by the time of admission to our hospital
and remained nonambulatory at the time of discharge to a
rehabilitation hospital.
Myasthenia Gravis
Five patients had myasthenia gravis (Tables 1-3); 2 were newly
diagnosed during their ICU/ICP admission. All of them had
bulbar dysfunction, and 4 had limb weakness. One of the newly
diagnosed patients had a very high titer of acetylcholine recep-
tor (AChR) binding and modulating antibodies, and electro-
myography showed marked decrement on repetitive nerve
stimulation (>50%). The other newly diagnosed patient had
negative AChR and muscle-specific kinase (MuSK) antibodies,
but an edrophonium test was positive. None of the patients with
myasthenia gravis had a thymoma. Two patients required
Table 2. Diagnosis.
Time (d) from admit to Neurology consult
Diagnosis made by
ICU Diagnostic studies for patients newly diagnosed during or after ICU admission
1 AFM 1 d 0 N CSF—WBC 50 (79%L), Pr 65.4; MRI—Spinal cord, brainstem 2 AFM 0 d 0 N CSF—WBC 102 (64%L), Pr 32.8; MRI—Spinal cord, brainstem 3 AFM 1 d 0 N CSF—WBC 29 (79%L), Pr 60.3; MRI—Spinal cord 4 GBS 1 d 0 N CSF—WBC 0, Pr 34.3; EMG—Axonal and demyelinating sensorimotor
polyneuropathy 5 GBS 0 d 0 N CSF—WBC 2, Pr 21.7; MRI—Cauda equina 6 GBS 0 d 0 N CSF—WBC 2, Pr 127.8; MRI—Cauda equina; EMG—Demyelinating motor
polyneuropathy 7 GBS 0 d 0 N CSF—WBC 2, Pr 28.6; EMG—Axonal and demyelinating sensorimotor
polyneuropathy 8 GBS 0 d 0 N CSF—WBC2, Pr 132 9 GBS 22 d 7 N CSF—WBC 0, Pr 16.9; EMG—Acute motor axonal neuropathy 10 CIDP 2 mo 0 N CSF—WBC 16 (92%L), Pr 337.9; MRI–cranial nerves, C/T/L nerve roots;
EMG–Demyelinating and axonal radiculoneuropathy; sural nerve biopsy—inflammation, demyelination, onion bulbs
11 MG 3 d 0 N AChR Bi ¼ 1401, AChR M ¼ 92%, striational (–), MuSK (–); EMG— Decrement with repetitive stimulation
12 MG N/A 0 N 13 MG N/A 0 N 14 MG 0 d 0 Y AChR Bi ¼ 0, AChR Bl ¼ 14%, AChR M ¼ 14%, MuSK (–) 15 MG N/A 0 N 16 CMS 2 y 0 N MRI—Posterolateral cervical spinal cord, lactate peak left BG; EMG—
Decrement with low-frequency stimulation; 2 pathogenic heterozygous recessive variants in ChAT gene in trans
17 IB 0 d 1 Y CSF—WBC 0, Pr 24; MRI—Lactate peak in left lentiform nucleus; EMG— Presynaptic NMJ disorder
18 IB 0 d 0 N CSF—WBC 4, Pr 79.2; EMG—Complex findings consistent with presynaptic NMJ disorder
19 HypoPP N/A 0 N 20 HypoPP 1 d 1 N 21 HypoPP 1 d 0 N CSF—WBC 2, Pr 28.3 22 Rhabdo 40 d 0 N CK—162920; Hemizygous pathogenic variant in DMD gene 23 Rhabdo 5.5 mo 0 N CK—72403; CSF—WBC 2, Pr 19.1; Muscle biopsy—Myofiber necrosis;
heterozygous splice site variant and exon deletion in LPIN1 gene in trans 24 Rhabdo N/A N/A N CK—105000
Abbreviations: AChR Bi, acetylcholine receptor binding antibody (nmol/L); AChR Bl, acetylcholine receptor blocking antibody; AChR M, acetylcholine receptor modulating antibody; AFM, acute flaccid myelitis; BG, basal ganglia; ChAT, choline acetyltransferase; CIDP, chronic inflammatory demyelinating polyneuropathy; CK, creatine kinase; CMS, congenital myasthenic syndrome; CSF, cerebrospinal fluid; C/T/L, cervical/thoracic/lumbar; DMD, Duchenne muscular dystrophy; EMG, electromyography; GBS, Guillain-Barre syndrome; HypoPP, hypokalemic periodic paralysis; IB, infant botulism; L, lymphocytes; MG, myasthenia gravis; MRI, magnetic resonance imaging with site of abnormalities listed; MuSK, muscle-specific kinase; NMJ, neuromuscular junction; Pr, protein (mg/dL); rhabdo, rhabdomyolysis; WBC, white blood cell (cells/mm3).
20 Journal of Child Neurology 35(1)
respiratory support, including endotracheal intubation. All
patients received pyridostigmine. Three patients were addition-
ally treated with plasmapheresis, 2 with oral corticosteroids,
and 1 each with intravenous corticosteroids and intravenous
immunoglobulin. Both of the patients who required intubation
during their admission developed pneumonia and a urinary
tract infection. Patients with myasthenia gravis spent a median
of 5 days in the hospital, 2 of which were spent in the ICU/ICP.
At the time of discharge, 2 patients were ambulatory with
limitations, and 3 were fully ambulatory. Three patients were
readmitted to the hospital 3, 4, and 7 times in the first year after
their first admission; 1, 4, and 6 of these admissions required
ICU/ICP level care, respectively.
An additional 10 patients with myasthenia gravis were
admitted to the ICU during the 11 years of our review for
observation after thymectomy. These patients are not described
further given they were admitted to the ICU solely for post-
operative monitoring.
One patient had congenital myasthenic syndrome (Tables 1-3).
He had ptosis, ophthalmoplegia, diffuse hypotonia, and axial
and limb weakness. He was admitted to the ICU for respiratory
distress and recurrent apnea; he had a pre-existing tracheost-
omy. He remained undiagnosed at the time of discharge.
Ongoing evaluation was notable for electromyography with
marked decrement on repetitive nerve stimulation. Genetic
testing revealed compound heterozygous pathogenic mutations
of the choline acetyltransferase (ChAT) gene, resulting in a
presynaptic form of congenital myasthenic syndrome. He was
treated with pyridostigmine with some functional improve-
ment. He resides in a residential facility and was readmitted
to the ICU twice during the first year after his initial admission;
both readmissions were for recurrent apnea and occurred prior
to diagnosis and initiation of pyridostigmine.
Infant Botulism
Two infants had botulism (Tables 1-3). Both had limb weak-
ness and one had bulbar dysfunction. Both infants required
intubation. In both infants, electromyography was notable for
a presynaptic neuromuscular junction defect, and botulism test-
ing returned positive after the infants had been discharged from
the hospital; both had been empirically treated with botulism
immunoglobulin. One infant developed pneumonia. The
infants remained in the hospital for almost 2 weeks, half of
which was spent in the ICU/ICP. Both infants were discharged
to home, and neither was readmitted to the hospital during the
first year after their initial presentation.
Disorders of Muscle
Hypokalemic Periodic Paralysis
1-3); 2 were newly diagnosed during their admission. All of
them presented with leg weakness and inability to walk; none
had bulbar dysfunction or respiratory distress. All 3 patients
required ICU/ICP-level care for intravenous potassium reple-
tion. ICU/ICP stay was brief and uncomplicated. All 3 patients
were ambulatory at baseline and were discharged to home with
no deficits. One patient was readmitted to the hospital, but not
to the ICU/ICP, during the first year after his initial admission.
Rhabdomyolysis
neuromuscular disorder (Tables 1-3). Two of them were newly
diagnosed based on testing sent during their admission, 1 with
Duchenne muscular dystrophy and the other with LPIN1 defi-
ciency. The other patient had a known diagnosis of phospho-
glycerate kinase deficiency. One patient had…
Dana B. Harrar, MD, PhD1,2 , Basil T. Darras, MD1, and Partha S. Ghosh, MD1
Abstract
Background: The neuromuscular disorders encountered in the pediatric intensive care unit (PICU) encompass a broad spectrum of pathologies. These include acute disorders (eg, Guillain-Barre syndrome), acute-on-chronic disorders (eg, myas- thenia gravis), progressive disorders (eg, muscular dystrophy), and disorders that develop in the PICU (eg, critical illness myo- pathy/polyneuropathy). Familiarity with the presenting features of these disorders is of paramount importance in facilitating timely diagnosis. Methods: We conducted a retrospective review of the medical records of patients admitted to the PICU or Inter- mediate Care Program (ICP) at a single tertiary children’s hospital from 2006 to 2017 with an acute or acute-on-chronic neu- romuscular disorder. We did not include patients with a known progressive neuromuscular disorder or critical illness myopathy/ polyneuropathy. Results: Twenty-four patients were admitted to the PICU/ICP with acute or acute-on-chronic neuromuscular disorders. Diagnosis and indication for ICU/ICP admission were Guillain-Barre syndrome (n¼ 6; respiratory failure: 3, respiratory monitoring: 2, autonomic instability: 1), myasthenia gravis (n ¼ 5; airway clearance: 3, respiratory failure: 2), acute flaccid myelitis (n ¼ 3; respiratory failure: 2, respiratory monitoring: 1), periodic paralysis (n ¼ 3; intravenous potassium replacement), rhab- domyolysis (n ¼ 3; monitoring for electrolyte derangements), infant botulism (n ¼ 2; respiratory failure), chronic demyelinating polyneuropathy (n ¼ 1; respiratory failure), and congenital myasthenic syndrome (n ¼ 1; apnea). No patients were admitted to the PICU/ICP with a diagnosis of tick paralysis, acute intermittent porphyria, or inflammatory myopathy. Conclusions: Although acute and acute-on-chronic neuromuscular disorders are encountered relatively rarely in the PICU, familiarity with the presenting features of these disorders is important in facilitating timely diagnosis. This, in turn, enables the institution of effective management strategies, thereby avoiding complications associated with diagnostic delays.
Keywords neuromuscular disorder, acute flaccid myelitis, inflammatory demyelinating polyneuropathy, myasthenia, botulism, periodic paralysis, rhabdomyolysis
Received May 4, 2019. Received revised July 8, 2019. Accepted for publication July 30, 2019.
The neuromuscular disorders encountered in the pediatric
intensive care unit (PICU) encompass a broad spectrum of
pathologies.1 These include acute disorders, often seen in pre-
viously healthy patients, such as Guillain-Barre syndrome and
infant botulism, acute-on-chronic disorders such as myasthenia
gravis and periodic paralysis, progressive disorders such as the
inherited myopathies and muscular dystrophies, and disorders
that develop in the intensive care unit in the form of critical
illness myopathies and polyneuropathies. The differential diag-
nosis of neuromuscular problems in the critically-ill neonate
and child was outlined in 2004 by Darras and Jones based on 24
years of experience in the electromyography laboratory at a
tertiary children’s hospital.2 Yates et al3 has described the out-
come of patients with progressive neuromuscular disorders
admitted to a pediatric intensive care unit, including patients
with spinal muscular atrophy, muscular dystrophies,
noninflammatory myopathies, and congenital and hereditary
neuropathies, whereas Banwell et al4 reported the incidence
and characteristics of weakness developing in critically-ill
patients in the pediatric intensive care unit. To our knowledge,
the frequency with which pediatric intensivists and intensive
care neurologists are likely to encounter acute and acute-on-
chronic neuromuscular disorders has not been reported. Here,
1 Department of Neurology, Boston Children’s Hospital, Boston, MA, USA 2 Division of Neurology, Children’s National Medical Center, Washington, DC,
USA
Corresponding Author:
Dana B. Harrar, MD, PhD, Division of Neurology, Children’s National Medical
Center, Washington, DC 20010, USA.
Email: [email protected]
acute and acute-on-chronic neuromuscular disorders in the
Pediatric Intensive Care Unit and Intermediate Care Program
at a tertiary children’s hospital.
Material and Methods
We conducted a retrospective review of the medical records of
patients admitted to the PICU and Intermediate Care Program (ICP)
at a tertiary children’s hospital between August 2006 and September
2017 for one of the following acute or acute-on-chronic neuromuscu-
lar disorders: acute flaccid myelitis, acute intermittent porphyria,
chronic inflammatory demyelinating polyneuropathy, congenital
myasthenic syndrome, Guillain-Barre syndrome, infant botulism,
inflammatory myopathy, myasthenia gravis, periodic paralysis, rhab-
domyolysis, and tick paralysis. The Intermediate Care Program pro-
vides a level of care intermediate between that of the ICU and the
general neurology ward. Patients were identified by searching the
electronic medical record using a centralized repository of clinical
data based on ICD-9 and -10 codes. For patients admitted to the ICU
or ICP multiple times during this 11-year period, we reviewed the first
admission to the ICU/ICP in detail and recorded the number of sub-
sequent admissions to the hospital during the first year after the initial
ICU/ICP admission but did not review subsequent admissions in
detail. We reviewed the patients’ charts for demographic information,
clinical characteristics, diagnostic investigations, treatment, and clin-
ical course. We did not include patients with known progressive dis-
orders, for example, spinal muscular atrophy, muscular dystrophy, and
noninflammatory myopathies, and disorders that develop in the inten-
sive care unit as a result of critical illness, for example, critical illness
myopathy and critical illness polyneuropathy, given that these cohorts
have been described previously.3,4 Study data were collected and
managed using Research Electronic Data Capture (REDCap) tools
hosted at Boston Children’s Hospital.5 We used descriptive statistics
as needed. All procedures performed in studies involving human
participants were in accordance with the ethical standards of the
Institutional Research Ethics Board of Boston Children’s Hospital
(IRB-P00024179). Consent was not required.
Results
Twenty-four patients were admitted to the Pediatric Intensive
Care Unit or Intermediate Care Program with an acute or acute-
on-chronic neuromuscular disorder between August 2006 and
September 2017. Approximately 3100 patients are admitted to
our pediatric intensive care unit each year, indicating that
admissions for acute and acute-on-chronic neuromuscular dis-
orders account for less than 0.1% of PICU admissions per year.
Diagnoses were as follows: Guillain-Barre syndrome (n ¼ 6),
myasthenia gravis (n ¼ 5), acute flaccid myelitis (n ¼ 3),
periodic paralysis (n ¼ 3), rhabdomyolysis (n ¼ 3), infant
botulism (n ¼ 2), chronic inflammatory demyelinating poly-
neuropathy (n¼ 1), and congenital myasthenic syndrome (n¼ 1).
No patients were admitted to the PICU or ICP within the 11-year
study period with a diagnosis of acute intermittent porphyria,
inflammatory myopathy, or tick paralysis. Nineteen (79%)
patients presented with a previously undiagnosed neuromuscular
disorder, 14 (74%) of whom were diagnosed prior to discharge.
Almost half of the patients in our cohort required ICU-level care
for invasive or noninvasive respiratory support, and respiratory
indications were the most common reason for admission to the
ICU/ICP (n ¼ 17, 71%). A majority of the neuromuscular
disorders encountered in our ICU over the past 11 years have
treatment options that, when initiated promptly, target the
underlying pathophysiologic process; in keeping with this, 17
(71%) patients were treated with potentially disease-modifying
therapeutics. One-third (n ¼ 8) of the patients in our cohort
experienced complications during their ICU admission, all of
whom were admitted for 9 days or more, and no patient died
as a complication of their neuromuscular disorder. We divide
these disorders below according to their localization in the
lower motor unit pathway.
Acute Flaccid Myelitis
Three patients had acute flaccid myelitis (Tables 1-3). All 3
patients were diagnosed in either 2014 or 2016, years during
which there was a national increase in reports of acute flaccid
myelitis. All of them had axial and limb weakness and
depressed deep tendon reflexes in the affected limbs. Cere-
brospinal fluid analysis demonstrated pleocytosis with a lym-
phocytic predominance in 3/3 patients and an elevated protein
in 2/3 patients; testing of the serum and cerebrospinal fluid for
infectious agents, including enterovirus, was negative. Neuroi-
maging revealed hyperintensity of the central gray matter of the
spinal cord in 3/3 patients, hyperintensity of the dorsal brain-
stem in 2/3 patients, and abnormal enhancement of the cauda
equina in 1/3 patients. Two patients required respiratory sup-
port, including one who required a tracheostomy. All patients
received intravenous immunoglobulin infusions. Additional
treatments included plasmapheresis (n ¼ 2), corticosteroids
(n ¼ 2), and fluoxetine (n ¼ 1), none of which are currently
favored treatment approaches.6 Two patients had a prolonged
(2 weeks) ICU/ICP admission, one complicated by
pneumonia and the other by tracheitis. At the time of dis-
charge, 2 patients were nonambulatory and 1 was ambulatory
with limitations. All 3 patients were discharged to a rehabi-
litation hospital.
Guillain-Barre Syndrome
them had a progressive ascending paralysis of the limbs. Cere-
brospinal fluid analysis revealed albuminocytologic dissocia-
tion in 2/6 patients at 5 and 14 days after symptom onset.
Cerebrospinal fluid analysis in the remaining 4 patients
revealed a normal protein level; in all 4 patients, cerebrospinal
fluid analysis was carried out within the first week after symp-
tom onset. Spine MRI revealed abnormal enhancement of the
cauda equina in 2/5 patients. Electromyography revealed
demyelinating polyneuropathy (n ¼ 1), motor axonal
18 Journal of Child Neurology 35(1)
neuropathy (n ¼ 1), or a mixed axonal and demyelinating
polyneuropathy (n ¼ 2). Three patients required respiratory
support, including 2 who required tracheostomy. All 6 patients
received intravenous immunoglobulin; additional treatments
included plasmapheresis in 3 patients and corticosteroids in
1 patient for concomitant systemic lupus erythematosus (SLE).
These patients spent a median of 24 days in the hospital with a
median of 11 days in the ICU/ICP. Complications during
admission included: pneumonia (n ¼ 3), Clostridium difficile
colitis (n ¼ 2), pneumothorax (n ¼ 1), pressure ulcer (n ¼ 1),
and UTI (n ¼ 1). Five patients were ambulatory at baseline; at
the time of discharge, 3 were no longer ambulatory and 2 were
ambulatory with limitations. Four patients were discharged to a
rehabilitation hospital, while the remaining 2 were discharged
home. The patient with acute motor axonal neuropathy was
readmitted to the hospital 8 times in the first year after his
initial ICU/ICP admission for complications related to SLE;
6 of these admissions required ICU/ICP-level care. None of
the remaining patients were re-admitted to the hospital in the
first year after admission for Guillain-Barre syndrome.
Chronic Inflammatory Demyelinating Polyneuropathy
neuropathy (CIDP) (Tables 1-3). She was admitted to the ICU
due to worsening axial and limb weakness. In addition, she
developed respiratory and bulbar dysfunction and required
intubation for almost 2 weeks. Cerebrospinal fluid analysis
revealed mild pleocytosis and a very high protein level. Spine
MRI showed diffuse thickening and enhancement of the
Table 1. Presenting Symptoms, Treatment, and Clinical Course.
Patient Age
Respiratory support Treatment
ICU days Complications
1 1/M AFM RD B; N; UE/LE; areflexia Tracheostomy Steroids, IVIG, fluoxetine
57 Tracheitis
2 4/M AFM RD B; N; UE/LE; areflexia Intubation Steroids, IVIG, PLEX 14 PNA 3 5/M AFM RM N; UE/LE; areflexia None Steroids, IVIG, PLEX 1 4 6/M GBS Autonomic EOM; Fa; B; UE/LE;
areflexia Tracheostomy Steroids, IVIG, PLEX 46 PNA, pressure ulcer,
C diff 5 16/F GBS RM Ptosis; UE/LE; areflexia None IVIG 3 6 17/M GBS RF Fa; B; UE/LE; S; areflexia BiPAP IVIG, PLEX 22 PNA, PTX, UTI 7 16/M GBS RF P; ptosis; B; UE/LE; S;
areflexia Tracheostomy IVIG, PLEX 28
8 6/F GBS RM B; N; UE/LE; areflexia None IVIG 2 9 18/M GBS RF UE/LE Tracheostomy* IVIG 11 PNA, SIADH, C diff 10 9/F CIDP RF Ptosis; EOM; B; UE/LE;
areflexia Intubation Steroids, IVIG, PLEX 15
11 13/F MG RD EOM; Fa; B; UE/LE Intubation Steroids, IVIG, Pyrido
27 PNA, UTI
12 14/F MG AC Ptosis; EOM; Fa; B; N; UE/LE
None PLEX, Pyrido 2
13 18/F MG AC Ptosis; EOM; B; UE/LE None Steroids, PLEX, Pyrido
2
14 11/F MG RF Ptosis; EOM; B; UE/LE Intubation PLEX, Pyrido 9 PNA, UTI 15 16/F MG AC Ptosis None Pyrido 1 16 0/M CMS RD P; UE/LE Tracheostomy* Supportive care 7 17 0/M IB RF B; N; UE/LE Intubation Botulism Ig 9 PNA 18 0/M IB RD B; UE/LE Intubation Botulism Ig 6 19 15/M HypoPP K repletion UE/LE None K repletion 2 20 16/M HypoPP K repletion None None K repletion 1 21 14/M HypoPP K repletion UE/LE None K repletion 1 22 2/M Rhabdo Electrolyte Gait abnormality None Supportive care 1 23 1/M Rhabdo Electrolyte UE/LE None Steroids, HCO3
fluids 5
Abbreviations: AC, airway clearance; AFM, acute flaccid myelitis; B, bulbar weakness; BiPAP, bilevel positive airway pressure; C diff, Clostridium difficile infection; CIDP, chronic inflammatory demyelinating polyneuropathy; CMS, congenital myasthenic syndrome; EOM, extraocular muscle weakness; F, female; Fa, facial weakness; GBS, Guillain-Barre syndrome; HCO3, bicarbonate; HypoPP, hypokalemic periodic paralysis; IB, infant botulism; Ig, immunoglobulin; IVIG, intravenous immunoglobulin; K, potassium; LE, lower extremity weakness; M, male; MG, myasthenia gravis; N, neck weakness; P, pupillary abnormalities; PLEX, plasmapheresis; PNA, pneumonia; PTX, pneumothorax; Pyrido, pyridostigmine; RD, respiratory distress; RF, respiratory failure; rhabdo, rhabdomyolysis; RM, respiratory monitoring; S, sensory abnormalities; SIADH, syndrome of inappropriate diuretic hormone secretion; UE, upper extremity weakness; UTI, urinary tract infection. *tracheostomy at baseline.
Harrar et al 19
linating and axonal features, and sural nerve biopsy showed
mild inflammation, focal demyelination, and the presence of
onion bulbs. An extensive infectious and paraneoplastic
work-up was negative. She was treated with intravenous
immunoglobulin, plasmapheresis, and intravenous corticos-
teroids and was then maintained on intravenous immunoglo-
bulin infusions and oral corticosteroids as an outpatient. She
spent 2 weeks in the ICU. She was ambulatory at baseline but
was nonambulatory by the time of admission to our hospital
and remained nonambulatory at the time of discharge to a
rehabilitation hospital.
Myasthenia Gravis
Five patients had myasthenia gravis (Tables 1-3); 2 were newly
diagnosed during their ICU/ICP admission. All of them had
bulbar dysfunction, and 4 had limb weakness. One of the newly
diagnosed patients had a very high titer of acetylcholine recep-
tor (AChR) binding and modulating antibodies, and electro-
myography showed marked decrement on repetitive nerve
stimulation (>50%). The other newly diagnosed patient had
negative AChR and muscle-specific kinase (MuSK) antibodies,
but an edrophonium test was positive. None of the patients with
myasthenia gravis had a thymoma. Two patients required
Table 2. Diagnosis.
Time (d) from admit to Neurology consult
Diagnosis made by
ICU Diagnostic studies for patients newly diagnosed during or after ICU admission
1 AFM 1 d 0 N CSF—WBC 50 (79%L), Pr 65.4; MRI—Spinal cord, brainstem 2 AFM 0 d 0 N CSF—WBC 102 (64%L), Pr 32.8; MRI—Spinal cord, brainstem 3 AFM 1 d 0 N CSF—WBC 29 (79%L), Pr 60.3; MRI—Spinal cord 4 GBS 1 d 0 N CSF—WBC 0, Pr 34.3; EMG—Axonal and demyelinating sensorimotor
polyneuropathy 5 GBS 0 d 0 N CSF—WBC 2, Pr 21.7; MRI—Cauda equina 6 GBS 0 d 0 N CSF—WBC 2, Pr 127.8; MRI—Cauda equina; EMG—Demyelinating motor
polyneuropathy 7 GBS 0 d 0 N CSF—WBC 2, Pr 28.6; EMG—Axonal and demyelinating sensorimotor
polyneuropathy 8 GBS 0 d 0 N CSF—WBC2, Pr 132 9 GBS 22 d 7 N CSF—WBC 0, Pr 16.9; EMG—Acute motor axonal neuropathy 10 CIDP 2 mo 0 N CSF—WBC 16 (92%L), Pr 337.9; MRI–cranial nerves, C/T/L nerve roots;
EMG–Demyelinating and axonal radiculoneuropathy; sural nerve biopsy—inflammation, demyelination, onion bulbs
11 MG 3 d 0 N AChR Bi ¼ 1401, AChR M ¼ 92%, striational (–), MuSK (–); EMG— Decrement with repetitive stimulation
12 MG N/A 0 N 13 MG N/A 0 N 14 MG 0 d 0 Y AChR Bi ¼ 0, AChR Bl ¼ 14%, AChR M ¼ 14%, MuSK (–) 15 MG N/A 0 N 16 CMS 2 y 0 N MRI—Posterolateral cervical spinal cord, lactate peak left BG; EMG—
Decrement with low-frequency stimulation; 2 pathogenic heterozygous recessive variants in ChAT gene in trans
17 IB 0 d 1 Y CSF—WBC 0, Pr 24; MRI—Lactate peak in left lentiform nucleus; EMG— Presynaptic NMJ disorder
18 IB 0 d 0 N CSF—WBC 4, Pr 79.2; EMG—Complex findings consistent with presynaptic NMJ disorder
19 HypoPP N/A 0 N 20 HypoPP 1 d 1 N 21 HypoPP 1 d 0 N CSF—WBC 2, Pr 28.3 22 Rhabdo 40 d 0 N CK—162920; Hemizygous pathogenic variant in DMD gene 23 Rhabdo 5.5 mo 0 N CK—72403; CSF—WBC 2, Pr 19.1; Muscle biopsy—Myofiber necrosis;
heterozygous splice site variant and exon deletion in LPIN1 gene in trans 24 Rhabdo N/A N/A N CK—105000
Abbreviations: AChR Bi, acetylcholine receptor binding antibody (nmol/L); AChR Bl, acetylcholine receptor blocking antibody; AChR M, acetylcholine receptor modulating antibody; AFM, acute flaccid myelitis; BG, basal ganglia; ChAT, choline acetyltransferase; CIDP, chronic inflammatory demyelinating polyneuropathy; CK, creatine kinase; CMS, congenital myasthenic syndrome; CSF, cerebrospinal fluid; C/T/L, cervical/thoracic/lumbar; DMD, Duchenne muscular dystrophy; EMG, electromyography; GBS, Guillain-Barre syndrome; HypoPP, hypokalemic periodic paralysis; IB, infant botulism; L, lymphocytes; MG, myasthenia gravis; MRI, magnetic resonance imaging with site of abnormalities listed; MuSK, muscle-specific kinase; NMJ, neuromuscular junction; Pr, protein (mg/dL); rhabdo, rhabdomyolysis; WBC, white blood cell (cells/mm3).
20 Journal of Child Neurology 35(1)
respiratory support, including endotracheal intubation. All
patients received pyridostigmine. Three patients were addition-
ally treated with plasmapheresis, 2 with oral corticosteroids,
and 1 each with intravenous corticosteroids and intravenous
immunoglobulin. Both of the patients who required intubation
during their admission developed pneumonia and a urinary
tract infection. Patients with myasthenia gravis spent a median
of 5 days in the hospital, 2 of which were spent in the ICU/ICP.
At the time of discharge, 2 patients were ambulatory with
limitations, and 3 were fully ambulatory. Three patients were
readmitted to the hospital 3, 4, and 7 times in the first year after
their first admission; 1, 4, and 6 of these admissions required
ICU/ICP level care, respectively.
An additional 10 patients with myasthenia gravis were
admitted to the ICU during the 11 years of our review for
observation after thymectomy. These patients are not described
further given they were admitted to the ICU solely for post-
operative monitoring.
One patient had congenital myasthenic syndrome (Tables 1-3).
He had ptosis, ophthalmoplegia, diffuse hypotonia, and axial
and limb weakness. He was admitted to the ICU for respiratory
distress and recurrent apnea; he had a pre-existing tracheost-
omy. He remained undiagnosed at the time of discharge.
Ongoing evaluation was notable for electromyography with
marked decrement on repetitive nerve stimulation. Genetic
testing revealed compound heterozygous pathogenic mutations
of the choline acetyltransferase (ChAT) gene, resulting in a
presynaptic form of congenital myasthenic syndrome. He was
treated with pyridostigmine with some functional improve-
ment. He resides in a residential facility and was readmitted
to the ICU twice during the first year after his initial admission;
both readmissions were for recurrent apnea and occurred prior
to diagnosis and initiation of pyridostigmine.
Infant Botulism
Two infants had botulism (Tables 1-3). Both had limb weak-
ness and one had bulbar dysfunction. Both infants required
intubation. In both infants, electromyography was notable for
a presynaptic neuromuscular junction defect, and botulism test-
ing returned positive after the infants had been discharged from
the hospital; both had been empirically treated with botulism
immunoglobulin. One infant developed pneumonia. The
infants remained in the hospital for almost 2 weeks, half of
which was spent in the ICU/ICP. Both infants were discharged
to home, and neither was readmitted to the hospital during the
first year after their initial presentation.
Disorders of Muscle
Hypokalemic Periodic Paralysis
1-3); 2 were newly diagnosed during their admission. All of
them presented with leg weakness and inability to walk; none
had bulbar dysfunction or respiratory distress. All 3 patients
required ICU/ICP-level care for intravenous potassium reple-
tion. ICU/ICP stay was brief and uncomplicated. All 3 patients
were ambulatory at baseline and were discharged to home with
no deficits. One patient was readmitted to the hospital, but not
to the ICU/ICP, during the first year after his initial admission.
Rhabdomyolysis
neuromuscular disorder (Tables 1-3). Two of them were newly
diagnosed based on testing sent during their admission, 1 with
Duchenne muscular dystrophy and the other with LPIN1 defi-
ciency. The other patient had a known diagnosis of phospho-
glycerate kinase deficiency. One patient had…
Related Documents
