|Year : 2022 | Volume
| Issue : 1 | Page : 44-46
Isolated unilateral palatal palsy secondary to COVID-19 infection in a child
Dwarkesh Valdoriya, Abhijeet Botre, Prashant Udavant
Department of Pediatrics, Ruby Hall Clinic, Pune, Maharashtra, India
|Date of Submission||20-Jun-2021|
|Date of Decision||16-Jan-2022|
|Date of Acceptance||28-Jan-2022|
|Date of Web Publication||25-Feb-2022|
Dr. Dwarkesh Valdoriya
Department of Pediatrics, Ruby Hall Clinic, 40, Sasson Road, Pune - 411 001, Maharashtra
Source of Support: None, Conflict of Interest: None
Background: Unilateral acquired isolated palatal paralysis is a very rare entity seen in children. It usually occurs due to isolated involvement of the pharyngeal branch of the vagus nerve. The definite etiopathogenesis is still unclear, but postinfectious immune-associated cranial mono-neuropathy is frequently postulated as plausible cause. We report an Indian girl who presented with isolated right palatal palsy following a coronavirus disease 2019infection. To the best of our knowledge, this has never been described in the literature before. Clinical Description: A 7.5-year-old girl child presented with nasal twang of voice and nasal regurgitation of liquids mainly from the right side of her mouth for 7 days. There was no evidence of any other neurological or systemic involvement. There was no history suggestive of any of the common causes usually attributed to palataopharyngeal palsy. Examination revealed right palatal palsy with deviation of the uvula to the left confirming lower motor neuron weakness of the pharyngeal branch of the vagus nerve. Management: Routine investigations excluding usual etiological causes were normal. The severe acute respiratory syndrome–corona virus 2 (SARS-CoV-2) immunoglobulin G antibody test was positive. The final diagnosis was postinfectious immune-mediated demyelinating isolated right palatal palsy. The child responded dramatically to a short pulse of methylprednisolone for 3 days and did not display any sequelae on follow-up. Conclusion: In the setting of the current pandemic, we recommend including SARS-CoV-2 serology in the routine workup of children presenting with isolated palatal palsy.
Keywords: Immune mediated, isolated palatal palsy, postinfectious, severe acute respiratory syndrome coronavirus 2
|How to cite this article:|
Valdoriya D, Botre A, Udavant P. Isolated unilateral palatal palsy secondary to COVID-19 infection in a child. Indian Pediatr Case Rep 2022;2:44-6
|How to cite this URL:|
Valdoriya D, Botre A, Udavant P. Isolated unilateral palatal palsy secondary to COVID-19 infection in a child. Indian Pediatr Case Rep [serial online] 2022 [cited 2022 May 27];2:44-6. Available from: http://www.ipcares.org/text.asp?2022/2/1/44/338476
Since the onset of the pandemic, many neurological manifestations have been described following severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infections. These include a wide spectrum of disorders such as encephalopathy, encephalitis, acute necrotizing encephalopathy, seizures, stroke, anosmia, ageusia, and Guillain − Barré syndrome (GBS). Many of these are the indirect consequence of immune phenomena following the infection, rather than the direct neurotrophic effects of viral invasion.
Unilateral acquired isolated palatal paralysis is a very rare entity described in children. It usually occurs due to isolated involvement of the pharyngeal branch of the vagus nerve. The precise etio-pathogenesis is still unclear. However, postinfectious immune-associated cranial mono-neuropathy has been frequently postulated as a plausible cause. Cranial nerve involvement like isolated ophthalmoplegia or concurrently with facial nerve palsy as a part of Miller Fisher syndrome, have been reported following coronavirus disease 2019 (COVID-19) infection in adults and children., We report a 7.5-year-old girl from India who presented with an isolated postinfectious/immune-mediated right palatal palsy after a COVID-19 infection. To the best of our knowledge, this has never been described in the scientific literature before.
| Clinical Description|| |
A 7.5-year-old previously healthy girl presented with sudden onset, nonprogressive nasal twang of voice and nasal regurgitation of liquids (mainly from the right side of her mouth) for 7 days, without increased drooling. There was no history of seizures, altered sensorium, abnormalities in the perception of smell, blurring of vision or development of squint, facial asymmetry, difficulty in chewing, loss of hearing, abnormal position of the tongue, any sudden weakness or inability to move the limbs, sensory impairment, or neck stiffness. There has been no history of any preceding febrile illness associated with rash, sore throat associated with painful or difficulty in swallowing or speaking, facial or cranial trauma, injury or operation of the throat, or joint involvement associated with swelling and painful restriction of movements. On probing further, it was revealed that two family members had been ill with COVID-19 infection 6 weeks earlier with fever and mild respiratory symptoms but had recovered uneventfully. The child herself had been asymptomatic. There was no significant history of similar complaints in the past or history of any previous hospitalization. The child was immunized. Early development was normal and she was performing adequately in school.
On examination, she had normal vitals. Her weight (23 kg) and height (120 cm) were between 25th and 50th centiles, as per age and gender. There were no abnormalities identified in the mouth or throat, presence of membrane, or significant cervical lymphadenopathy. Consciousness was preserved and her higher mental functions were normal. Salient central nervous system findings were decreased movement of the right palate with deviation of the uvula to the left on phonation. The sensation over the posterior pharyngeal wall was intact with the presence of a consensual gag reflex, indicative of a lower motor neuron (LMN) weakness of the pharyngeal branch of the vagus nerve [Figure 1]. Examination of the remaining cranial nerves did not reveal any dysfunction. There was no other motor focal deficit with normal tone, power, and deep-tendon reflexes of all four limbs. There were no cerebellar signs, involuntary movements, and signs suggestive of autonomic involvement, raised pressure, or meningeal irritation. Remaining systemic examination was unremarkable. The provisional clinical diagnosis kept was that of an isolated right LMN vagus nerve palsy. Considering the differential diagnosis of a structural lesion, or a postinfectious/immune-mediated etiology, neuroimaging, electrophysiology and blood investigations were planned.
|Figure 1: Isolated right palatal palsy with deviation of the uvula to the right side|
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| Management and Outcome|| |
The hemogram (hemoglobin 13.9 g/dl, total leukocyte count 7830/mm3 and platelet count 3.28 lacs/mm3), serum electrolytes (sodium 137 mmol/L, potassium 4.2 mmol/L, calcium 10.3 mg/dl, and phosphorus 6.6 mg/dl), Vitamin B12 levels (440 pg/ml), and inflammatory markers (high sensitive C-reactive protein <0.04 mg/dl and erythrocyte sedimentation rate 10 mm at end of 1 h) were normal. Magnetic resonance imaging of the brain (with dedicated lower cranial nerves imaging and cerebellopontine angle) revealed no focal lesion or external compression. A lumbar puncture was planned to rule out any indolent intracranial infections. The cerebrospinal fluid (CSF) analysis was normal with a total cell count of 2 leukocytes per mm3 (100% lymphocytes), glucose level of 82 mg/dl, and protein level of 12.8 mg/dl. The nerve conduction studies for motor and sensory nerves of the upper and lower limbs showed normal conduction velocity and compound motor action potential amplitudes, thus ruling out any evidence of subclinical polyneuropathy. In the setting of the pandemic, and since there was a positive history of COVID-19 infection in the family, the child's SARS-CoV-2 immunoglobulin G (IgG) antibody test was sent. This was positive (1.53U/ml, normal <1.1U/ml).
Based on the history (duration between COVID infection in the household and appearance of symptoms in the child), examination, and work-up that ruled out structural brain/brainstem lesions, infectious/vascular causes, and polyneuropathy, a clinical diagnosis of postinfectious or immune-mediated demyelinating isolated right palatal palsy was established. A pulse course of intravenous methylprednisolone therapy (30 mg/kg/dose) was given for 3 days. Within 4 days, there was marked clinical improvement. The nasal twang disappeared, nasal regurgitation of liquids subsided, and the uvula became central in position; hence, the need for further oral steroids was not considered necessary. The child was discharged and kept under follow-up. At the 2-month visit, she was asymptomatic and had no residual neurological deficit.
| Discussion|| |
Isolated acquired palatal palsy is a rare clinical entity that primarily affects males in their first or the second decade of life. Clinical manifestations include hypernasal speech (97%), nasal regurgitation (73%), and dysphagia (49%), mimicking brainstem lesions. There are two mechanisms that have been proposed related to etiopathogenesis. First, postinfectious cranial mononeuropathy due to an acute infection (mainly viral), as the relative immaturity of neural tissue and an increased prevalence of respiratory and gastrointestinal tract infections may lead to increased susceptibility in children. Second, ischemia results from a vascular insult to the roots of the IXth and Xth cranial nerves resulting in LM neuropathies that manifest as palatopharyngeal paralysis. The preservation of normal posterior pharyngeal sensations and an intact consensual gag reflex confirms that only the pharyngeal branch of the vagal nerve is involved. Earlier, a common differential diagnosis would have been postdiphtheritic syndrome with predominant palatal palsy, but the child was completely immunized (including the 5-year booster) so the likelihood was quite low, besides the absence of other typical clinical manifestations. Isolated palatal paralysis is usually idiopathic condition, but one must exclude other etiological causes like craniofacial trauma, operations (adenoidectomy), infections (herpes simplex virus, Coxsackie, Rubeola, Hepatitis A, Varicella, and Ebstein − Barr virus attribute to 20% of cases), neuromuscular disorder such as GBS, and vascular insults (i.e., an internal carotid artery aneurysm).
The fact that symptoms developed of palatopharyngeal palsy 6 weeks after her family members were symptomatic and she exhibited IgG antibodies against SARS-CoV-2 supports the typical time frame associated with postinfectious/demyelinating syndromes. We did not send CSF and serum investigations for the other viruses since there were no constitutional symptoms and the normal sepsis biomarkers and CSF reports precluded an acute central nervous system infection. A literature search of other reports of isolated cranial nerve palsies secondary to SARS-CoV-2 infection in children revealed the rare occurrence of 3rd and 7th cranial nerve either as a part of multisystem inflammatory syndrome in children, GBS or as an isolated phenomenon., We recommend that in the setting of a pandemic, routine SARS-CoV-2 serology testing should be included in the panel of other causes that need to be ruled out before labeling isolated palatal palsy as idiopathic in children. This is especially important given that timely initiation of immunosuppression with steroids such as methylprednisolone have a definitive therapeutic role in the management.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal patient identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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