Sobbing spasm

Pediatric criteria
Acta Pediátr Mex 2014;35:152-154.
Sobbing spasm
Matilde Ruíz-García
Head of Neurology Service
Instituto Nacional de Pediatría
Definition
Sobbing spasm is the most common non-epileptic paroxystic
phenomenon in infants and preschool age children; it is observed
in 5 to 7% of infants and preschool age children.1 It is secondary
to episodes of acute cerebral hypoxia precipitated by crying, pain,
surprise, or frustration. Following a deep inspiration a spasm is
produced, which stops respiration and conditions apnea. The
patient goes rigid, with cyanosis of lips and fingers and toes, or
flaccid and pallid.
It often begins between 6 and 12 months of life; in the large
majority of cases it disappears before 6 years of age. The greatest frequency occurs between one year and two years of age.2,3
Classification
Two types of sobbing spasm are recognized:
A. Cyanotic: episodes that begin in the course of crying due
to frustration, pain, or anger. After one or several respiratory
movements during crying, it is interrupted, the child goes into
apnea, and after a few seconds turns cyanotic, and may even
lose consciousness. Loss of consciousness may be associated
with generalized hypotonia or hypertonia with opisthotonos, and
subsequently short seizures. This sequence may be complete or
incomplete. In any case, recuperation is immediate. After a few
seconds the child regains full wakefulness. The total duration is
1-4 minutes.4,5
Four grades are distinguished:
Accepted: March, 2014
Correspondence
Grade 2. Crying, prolonged inspiration, apnea and acrocyanosis.
Dra. Matilde Ruíz García
Jefa del Servicio de Neurología
Clínica de Epilepsia del Instituto Nacional de Pediatría
matilderuizg@hotmail.com
Grade 3. Crying, prolonged inspiration, apnea, cyanosis, and
hypotonia or hypertonia.
This article must be quoted
Ruiz García M. Espasmo del sollozo. Acta Pediat Mex
2014;35:166-169.
Grade 1. Crying with prolonged inspiration and brief apnea.
152
Received: February, 2014
www.actapediatricademexico.org
Ruiz García M. Sobbing spasm
Grade 4. Crying, prolonged inspiration, apnea,
cyanosis, hypertonia and generalized clonic
seizures.
Recommended medical evaluation
B. Pallid or reflex anoxic seizure: following minor trauma (especially to the head) or a situation
of fear or surprise, the patient starts crying and
loses consciousness; the patient is pallid with
generalized hypotonia and may also present
clonic seizures of extremities. This is due to an
increased cardioinhibitory response, secondary
to an increase in vagal tone.
2. Physical examination.
In some cases, the two forms alternate in the
same patient. Some children with pallid spasms
will subsequently present infant juvenile syncope
as a hypervagotonic reflex reaction to situations
of stress.2,3
5. Electrocardiogram in case of pallid sobbing
spasm. To rule out arrhythmias or long QT
syndrome.
C. Mixed. Pallid and cyanotic sobbing spasms
may coexist in a patient, although it is uncommon.5
1. Keep calm.
Pathophysiology
Sobbing spasm has been related to a primitive
childhood respiratory reflex; it has a certain degree of family aggregation, because one in every
four children with sobbing spasm has a direct
family member who suffered it in infancy.6 Some
authors have related iron deficiency anemia to
the genesis of sobbing spasm and hemoglobin
below 8 g/100 mL has been reported in up to
23.5% of children with sobbing spasms. Iron deficiency may play a role in the pathophysiology
of sobbing spasm because iron is important for
catecholamine metabolism and the functioning
of neurotransmitters.7
It has been suggested that a delay in maturation
of brainstem myelination, measured through
brainstem evoked potentials, may influence the
development of sobbing spasm.6,8
1. Complete clinical history.
3. Blood biometry in case of suspected anemia.
4. An electroencephalogram should be performed if epilepsy is suspected, when there
is no evident triggering phenomenon, in patients under 6 months or over 6 years of age.
Management of sobbing spasm
2. Remove objects the child has in his or her
mouth.
3. Place the patient on his or her side and remove any objects with which he or she could
hit himself/herself.
4. Maintain a well-ventilated environment,
loosen the patient’s clothing.
5. Do not attempt to stop the spasm.
6. Speak softly.
7. When the spasm ends let the patient take a
short nap.
8. If the spasm is caused by pain offer consolation and relief.
If a child has several sobbing spasms a day behavioral management is probably inappropriate.
153
Volume 35, No. 2, March-April 2014
Acta Pediátrica de México
Some children use spasms as an inappropriate
form of communication.
Some authors recommend the use of piracetam
in patients with recurrent sobbing spasm.9
What should be avoided?
Antiepileptic treatment is not justified.
1. Resuscitation maneuvers.
Long-term follow-up suggests that sobbing
spasms are alleviated spontaneously. Patients
present a higher incidence of attention problems,
and in the case of pallid spasms, may present a
higher incidence of syncope.10
2. Striking or bathing the child with cold water.
There is a risk of bronchoaspiration and pulmonary complications.
3. Do not place objects in the child’s mouth, as
they can cause injury or suffocation.
REFERENCES
1.
Visser AM, Jaddoe VW, Arends LR, Tiemeier H, Hofman A,
Moll HA, Steegers EA, Breteler MM, Arts WF. Paroxysmal
disorders in infancy and their risk factors in a populationbased cohort: the Generation R Study. Dev Med Child
Neurol 2010;52:1014-20.
2.
Martinez A. Chapter 14: Trastornos paroxísticos no epilépticos en los primeros años. En: Campistol J. Neurología para
pediatras, enfoque y manejo práctico. Barcelona: Editorial
Médica Panamericana, 2011. pp. 191-202.
3.
Swaiman KF, Ashwal S, Ferreiro DM. Cap 65. Movement
Disorders. In: Pediatric Neurology; Principles and Practice.
Philadelphia: Elsevier, 2012. pp. 880-925.
4.
Yılmaz Ü, Serdaroğlu A, Gürkaş E, Hirfanoğlu T, Cansu A.
Childhood paroxysmal nonepileptic events. Epilepsy and
Behavior 2013;27:124-9.
5.
Malagon-J Trastornos paroxísticos no epilépticos durante
el sueño. Rev Neurol 2013;57(Suppl 1):S115-S123.
6.
Vurucu S, Karaoglu A, Paksu SM, Oz O, Yaman H, Gulgun
M, Babacan O, Unay B, Akin R. Breath-Holding Spells May
be Associated With Maturational Delay in Myelination of
Brain Stem. J Clin Neurophysiol. 2014;31:99-101.
7.
Fejerman N, Medina CS, Caraballo RN. Trastornos paroxísticos y síntomas episódicos no epilépticos. In: Fejerman N,
Fernández Álvarez E. Neurología Pediátrica. Buenos Aires:
Editorial Médica Panamericana, 2010. pp. 660-673.
8.
There is no specific medication; it is recommended to provide extensive information to the
family and use educational strategies for control
and inhibition of sobbing spasms.
Arslan H, Torun E, Akkan JC, Guler S, Bayraktar S. The Evaluation of Physiological and Biochemical Parameters and
the Autonomic Nervous Systems of Children with BreathHolding Spells. Neuropediatrics 2013 Dec 13. [Epub ahead
of print]
9.
Sawires H, Botrous O. Double-blind, placebo-controlled
trial on the effect of piracetam on breath-holding spells.
Eur J Pediatr. 2012;171:1063-7. doi: 10.1007/s00431-0121680-1.
If iron deficiency anemia is confirmed management with diet and ferrous sulfate is suggested.5,7
10. Olsen AL, Mathiasen R, Rasmussen NH, Knudsen FU. Longterm prognosis for children with breath-holding spells. Dan
Med Bull 2010;57:A4217.
4. It is very important not to confuse sobbing
spasm with other convulsive disorders; avoid
administering antiepileptic drugs as much as
possible.
When is medical reevaluation needed?
1. When episodes occur without a triggering
factor or during sleep.
2. In case of convulsive movements.
3. In case of onset before 5 months or after 6
years of age.
4. When there is diagnostic doubt.
The prognosis is good, sobbing spasm is not associated with subsequent development of epilepsy
or cognitive alterations.
Treatment
154