Journal of Neurological Sciences (Turkish)

Isolated neuropathies with possible viral or postviral etiologies may affect only one nerve such as nervus hypoglossus, recurrens or vagus, some others may affect both the 9^th and 10^th cranial nerves unilaterally causing isolated temporary paralysis of the pharynx that mimics diseases of the brain stem⁽¹⁾. Onset of isolated neuropathies is generally follows infections of upper respiratory tract like infectious mononucleosis and parvovirus B19, and is characterized by nasal reflux and/or dysphagia⁽⁷^,⁹⁾. Physical exam reveals unilateral paralysis of soft palate and descending of pharyngeal wall on the lesion side. Spontaneous recovery is observed after a few days or weeks. Similar characteristics such as acute onset, appearing in infacy (96%), predominance in males (79%), recent respiratory infection (35%) and an excellent prognosis for recovery (85%) have been described in the literature in patients with unilateral paralysis of the soft palate⁽²^,⁶⁾.

Paralyses of the both 9^th and 10^th cranial nerves were diagnosed in our patient since he had pressure and pain on his chest in addition to regurgitation, unilateral palatal paralysis and rhinolalia. Initial laboratory tests performed for existence of an infection and cranial MRI performed for a possible brain stem lesion did not reveal any abnormality. Existence of a motor neuron disease was excluded since deep tendon reflexes, muscle power of all four limbs and peripheral nerve conductance examined by EMG were normal. Depending on these findings, we diagnosed isolated idiopathic velopalatin palsy in our patient. Similarly, Gonzales et al reported sudden onset dysfunction of the 9 and 10. cranial nerves in a 5-year-old girl. The clinical course is favorable and the results of complementary investigations are normal. These authors reported an excellent prognosis with a high percentage of complete recovery and absence of recurrences in their patient⁽³⁾. According to our knowledge, this is the second case of an idiopathic unilateral velopalatin palsy.

The main problem is the extensive exploration undertaken to achieve etiological diagnosis. Extensive laboratory and clinical investigations should be performed since intracranial tumors, osteomyelitis, meningitis, brain stem infarct, demyelinizing diseases, motor neuron diseases, poliomyelitis, penetrating injuries, diabetes, syphilis, borrelia, Mycoplasma, Chlamydia, Varicella zoster, Ebstein-Barr virus, Cytomegalovirus, Rubeola, parotitidis, sarcoidosis or SLE may underlie lower cranial nerve paralyses⁽⁵^,⁸^,⁶^,³⁾. Diphtheria toxoid, which generally affects motor nerves, may lead to glossopharyngeal palsy via causing toxic neuropathy 2-8 weeks after the onset of diphtheria⁽⁴⁾.

Conclusion, idiopathic velopalatin palsy should be suspected in patients between 5 and 15 years of age who present with a sudden onset palsy of the IX and X cranial nerves and without any other symptoms. We would like to make an emphasis on the totally-recovering nature of this idiopathic and temporary disease which requires that detailed anamnesis be obtained and laboratory investigations be determined depending on clinical condition of the patient.

Received by: 25 December 2007
Revised by: 09 February 2008
Accepted: 07 March 2008

Journal of Neurological Sciences (Turkish)
2009, Volume 26, Number 2, Page(s) 248-250
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Spontaneously Recovered Idiopathic Unilateral Velopalatin Palsy
Aycan ÜNALP, Uran NEDRET, Ceyhun DİZDARER, Özcan TUĞRUL
Ministry of Health, Behcet Uz Pediatric Hospital, Dept. of Pediatric Neurology, Izmir, Turkey
Summary
Velopalatin palsy, is a rare entity in children which affects 9th and 10th cranial nerves. A 7-year-old boy presented with a complaint of regurgitation of fluids into the nois and rhinolalia. Shift of the uvula to the right side and descending of pharyngeal wall in the left side were detected in physical exam. The patient had pressure and pain on his chest. No abnormality had been found in telecardiography, electrocardiography, bacteriologic-viral serology tests, and cranial magnetic resonance imaging. The child recovered spontaneously after 2 weeks. The case has been presented here because of the scarce and benign nature of this disorder. How to cite this article: A. ÜNALP, U. NEDRET, C. DİZDARER, Ö. TUĞRUL: Spontaneously Recovered Idiopathic Unilateral Velopalatin Palsy. J Neurol Sci [Turk] 2009;26:248-250 How to cite this URL & PDF: A. ÜNALP, U. NEDRET, C. DİZDARER, Ö. TUĞRUL: Spontaneously Recovered Idiopathic Unilateral Velopalatin Palsy. J Neurol Sci [Turk] 2009 [cited 2009 June 19];26:248-250. Available from: http://jns.dergisi.org/text.php3?id=284 PDF: http://jns.dergisi.org/pdf/pdf_JNS_284.pdf E-mail of the corresponding author: aycanunalp@mynet.com	Top Summary Introduction Case Presentation Discussion References
Introduction
Isolated lower cranial neuropathies may have various etiologies⁽⁵^,⁶^,⁸⁾. Of these velopalatin palsy, is a rare entity in children which affects 9^th and 10^th cranial nerves. Nervus glossopharyngeus is a mixed-function cranial nerve that contains sensory, motor and parasympathetic fibers. Glossopharyngeal paralysis caused by lesions of the medulla and nerve root is generally accompanied by paralysis of another nerve. Nervus vagus also is a mixed-function cranial nerve that contains sensory, motor and parasympathetic fibers. Bilateral paralysis of vagal nerve results in loss of vomiting reflex, nasal regurgitation of fluids, difficulty in swallowing foods and nasal voice, while hoarseness and unilateral descending of pharyngeal wall are observed after unilateral paralysis of this nevre⁽³⁾. We aimed in this report to discuss the diagnosis and treatment of sudden, temporary and idiopathic velopalatin palsy, in the light of the literature.	Top Summary Introduction Case Presentation Disscussion References
Case Presentation
A 7-year-old boy presented to our hospital with a complaint of regurgitation of fluids into the nose. No pain, fever, or trauma was described before the onset of this symptom. Hoarseness, shift of the uvula to the right side and descending of the left side palate were detected in physical examination. The patient mentioned that he had pressure and pain on his chest a few days before these complaints, but no abnormality had been found in laboratory tests such as hemogram, blood glucose, sedimentation, CRP, liver and kidney functions, telecardiography, electrocardiography; no infection was detected by bacterologic and viral serology tests. No abnormality was detected in cranial MRI performed for visualizing brain stem lesions. Normal nerve conductance was obtained in upper limb EMG. The patient recovered spontaneously after 2 weeks and on 1 year following period no recurrence was detected.	Top Summary Introduction Case Presentation Disscussion References
Discussion
Isolated neuropathies with possible viral or postviral etiologies may affect only one nerve such as nervus hypoglossus, recurrens or vagus, some others may affect both the 9^th and 10^th cranial nerves unilaterally causing isolated temporary paralysis of the pharynx that mimics diseases of the brain stem⁽¹⁾. Onset of isolated neuropathies is generally follows infections of upper respiratory tract like infectious mononucleosis and parvovirus B19, and is characterized by nasal reflux and/or dysphagia⁽⁷^,⁹⁾. Physical exam reveals unilateral paralysis of soft palate and descending of pharyngeal wall on the lesion side. Spontaneous recovery is observed after a few days or weeks. Similar characteristics such as acute onset, appearing in infacy (96%), predominance in males (79%), recent respiratory infection (35%) and an excellent prognosis for recovery (85%) have been described in the literature in patients with unilateral paralysis of the soft palate⁽²^,⁶⁾. Paralyses of the both 9^th and 10^th cranial nerves were diagnosed in our patient since he had pressure and pain on his chest in addition to regurgitation, unilateral palatal paralysis and rhinolalia. Initial laboratory tests performed for existence of an infection and cranial MRI performed for a possible brain stem lesion did not reveal any abnormality. Existence of a motor neuron disease was excluded since deep tendon reflexes, muscle power of all four limbs and peripheral nerve conductance examined by EMG were normal. Depending on these findings, we diagnosed isolated idiopathic velopalatin palsy in our patient. Similarly, Gonzales et al reported sudden onset dysfunction of the 9 and 10. cranial nerves in a 5-year-old girl. The clinical course is favorable and the results of complementary investigations are normal. These authors reported an excellent prognosis with a high percentage of complete recovery and absence of recurrences in their patient⁽³⁾. According to our knowledge, this is the second case of an idiopathic unilateral velopalatin palsy. The main problem is the extensive exploration undertaken to achieve etiological diagnosis. Extensive laboratory and clinical investigations should be performed since intracranial tumors, osteomyelitis, meningitis, brain stem infarct, demyelinizing diseases, motor neuron diseases, poliomyelitis, penetrating injuries, diabetes, syphilis, borrelia, Mycoplasma, Chlamydia, Varicella zoster, Ebstein-Barr virus, Cytomegalovirus, Rubeola, parotitidis, sarcoidosis or SLE may underlie lower cranial nerve paralyses⁽⁵^,⁸^,⁶^,³⁾. Diphtheria toxoid, which generally affects motor nerves, may lead to glossopharyngeal palsy via causing toxic neuropathy 2-8 weeks after the onset of diphtheria⁽⁴⁾. Conclusion, idiopathic velopalatin palsy should be suspected in patients between 5 and 15 years of age who present with a sudden onset palsy of the IX and X cranial nerves and without any other symptoms. We would like to make an emphasis on the totally-recovering nature of this idiopathic and temporary disease which requires that detailed anamnesis be obtained and laboratory investigations be determined depending on clinical condition of the patient. Received by: 25 December 2007 Revised by: 09 February 2008 Accepted: 07 March 2008	Top Summary Introduction Case Presentation Discussion References
References
1) Auberge C, Ponsot G, Gayraud P et al. Les hemiparesies velopalatines isolees et acquises chez l'enfant. Archives Françaises de Pediatrie 1979; 36: 283-286. 2) Cuvellier JC, Cuisset JM, Nuyts JP, Vallée L. Acquired and isolated asymmetrical palatal palsy. Neuropediatrics 1998; 29: 324-325. 3) González Alvarez V, Costa Orvay JA, Guardia Camí MT, Garrido Romero R, Pineda Marfá M, Luaces Cubells C. [Idiopathic palatopharyngeal hemiparalysis] An Pediatr 2003; 59: 595-598. 4) Havaldar PV. Diphtheria in the eighties: experience in a south Indian district hospital. J Indian Med Assoc 1992; 90: 155-156. 5) Nakagawa H, Nagasao M, Kusuyama T, Fukuda H, Ogawa K. A case of glossopharyngeal zoster diagnosed by detecting viral specific antigen in the pharyngeal mucous membrane. J Laryngol Otol 2007; 121:163-165. 6) Pollack MA, Shprintzen RJ. Velopharingeal insufficiency in neurofibromatosis. Int J Pediatr Otorhinolaryngol 1981; 3: 257-262. 7) Soares-Fernandes JP, Maré R. Isolated velopalatine paralysis associated with parvovirus B19 infection. Arq Neuropsiquiatr 2006; 64: 603-605. 8) Soyal OB, Turan S, Durak P, Erdemli O. Transient palsy of peripheral cranial nerves following open heart surgery. Singapore Med J 2006; 47: 422-424. 9) Sugama S, Matsunaga T, Ito F et al. Transient unilateral, isolated hypoglossal nerve palsy. Brain and Development 1992; 14: 122-123. 10) Villarejo-Galende A, Camacho-Salas A, Penas-Prado M, García-Ramos R, Mendoza MC, Simón de las Heras R, Mateos-Beato F. [Unilateral isolated paralysis of the soft palate: a case report and a review of the literature]. Rev Neurol 2003; 36: 337-339.	Top Summary Introduction Case Presentation Discussion References
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