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Indian Pediatr 2011;48: 237-239 |
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Isolated Idiopathic Unilateral Paralysis of
Soft Palate and Pharynx |
Vishal Sondhi and Suprabha Kumari Patnaik
From Department of Pediatrics, Military Hospital, Ambala Cantt, India.
Correspondence to: Maj. Vishal Sondhi, Department of Pediatrics, Military Hospital,
Ambala Cantt, Haryana, India.
Received: May 30, 2009;
Initial review: June 22, 2009;
Accepted: November 23, 2009.
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Isolated unilateral palatal (velopalatopharyngeal) palsy is a clinical
rarity. We report this entity in a 10 year old boy, two weeks after an
episode of acute tonsillitis. The child was managed with a short course
of prednisolone with complete recovery in eleven days.
Key words: Palatal paralysis, Rhinolalia, Tonsillitis.
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Idiopathic unilateral palatal
paralysis is a rare disorder. Since its first description in 1976, 35
cases have been described [1]. The disorder typically affects boys at ages
of 2-3 years with sudden onset rhinolalia and nasal regurgitation of
fluids [2-5]. We report a 10-year-old boy with this illness.
Case Report
A previously well, immunized, 10-year-old boy,
presented with fever and sore throat of four days duration. Examination
revealed bilaterally inflamed tonsils. A throat swab was sent for
analysis, and the child was given injection benzathine penicillin
(intramuscularly; one dose) and acetaminophen (per orally). Throat swab
revealed group-A beta-hemolytic (GABH) streptococcal infection. Viral
studies could not be performed due to non-availability of facilities. The
child became asymptomatic on seventh day after presentation.
The child was again brought, two weeks from the onset
of first symptoms, with rhinolalia, nasal escape of fluids from the right
nostril, and dysphagia. There was no recurrence of fever, or sore throat.
Examination revealed deviation of uvula to right with absent gag reflex on
left side of pharyngeal wall (Fig 1). Rest of neurological
examination and laryngoscopy was normal. Cerebrospinal fluid examination
and magnetic resonance imaging (MRI) of brain and cervical spine revealed
no abnormality.
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Fig. 1 Uvula deviated to right on phonation.
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The unilateral paralysis of soft palate and pharynx was
thought to be idiopathic in origin. The child was started on oral
prednisolone (2mg/kg/day). The steroids were tapered from 6th day and
stopped on day 15. The symptoms started improving on day four of steroids
and the child was completely asymptomatic nine days after starting
steroids. The palatal palsy recovered completely by eleventh day from the
onset of symptoms. There was no recurrence of symptoms for two years that
the child was followed.
Discussion
We consider that vagus is the most probable nerve
affected. The mode of involvement of vagus is not known but two mechanisms
have been hypothesized: viral and vascular [5]. An infectious/
postinfectious cranial neuropathy seems likely as isolated palato-pharyngeal
palsy is well documented as a post diphtheritic complication. The
clinical features of diphtheria were not present in our patient and also,
he was completely vaccinated. There have been sporadic reports of palato-pharyngeal
palsy in children following infection with herpes, varicella, and
hepatitis A [3]. However, definitive evidence in the form of positive
viral serology/ culture could be demonstrated in only five of the twelve
cases with a febrile prodrome [3,5]. In our case, the child had acute
tonsillitis with a positive throat culture for GABH streptococci two weeks
prior to palatal palsy. The literature search did not reveal any
association between GABH streptococcal infection and palatal palsy.
However, co-infection with a virus cannot be ruled out since viral
culture/serological studies could not be carried out.
The second hypothesis has been proposed by Lapresle,
et al. [8]. They demonstrated the existence of ischemia in the roots
of the glossopharyngeal and vagus nerves. The cause of this ischemia is
not known and viral infection induced vasculitis cannot be ruled out.
Either of the aforementioned scenarios would lead to lower motor neuron
neuropathy manifesting as palatopharyngeal incompetence. The higher
incidence of the condition in childhood is possibly due to immature neural
tissue that is rendered more susceptible than in adults.
Other possibilities considered were Guillain-Barre
syndrome, vascular insults, posterior fossa tumors, syringobulbia, and
inflammatory diseases affecting brainstem nuclei. Detailed pharyngeal and
neurologic examination, evaluation of cerebrospinal fluid, and MRI of the
brain and upper cervical region were unremarkable. We consider the
paralysis in our case to be idiopathic because all the evaluations were
negative.
The prognosis of the disorder is excellent with
complete recovery in >85% cases [5], and only one recurrence reported till
date [7]. There is no specific treatment; oral glycerol and steroids have
been used and complete recovery has been reported even without any
treatment [3]. We managed the case with short course of steroids without
any side effects, and the child started improving from day four and the
recovery was complete by day eleven. Several reports suggest initiation of
recovery by tenth day and reversal of pharyngeal weakness by 4-7 weeks
without the use of steroids [3,5]. Nonetheless, further reports focusing
on the therapeutic aspect are desired, before a recommendation is made.
Contributors: Both the authors were involved in
diagnosis, management of the patient and in writing of the paper.
Funding: None.
Competing interests. None stated.
References
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temporary pharyngeal paralysis in childhood. Lancet. 1976;1:1047-9.
2. Villarejo-Galende A, Camacho-Salas A, Penas-Prado M,
Garcia-Ramos R, Mendoza MC, Simon de las Heras R, et al. Unilateral
isolated paralysis of the soft palate: a case report and a review of the
literature. Rev Neurol. 2003;36:337-9.
3. Prasad PL, Prasad AN, Patnaik SK. Unilateral palatal
palsy with viral hepatitis. Indian J Pediatr. 2007;74:1039-40.
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Acquired and isolated asymmetrical palatal palsy. Neuropediatrics.
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angiography. Contribution to the ischemic pathology of the cranial nerves.
Rev Neurol. 1980;136:787-91.
9. Auvin S, Cuvellier JC, Vallee L. Isolated recurrent palatal palsy in
a child. Neuropediatrics. 2003;34:278-9.
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