Gallego-Perez-Larraya, J. (Jaime)

Riverol, M. (Mario)

Dadun, University of Navarra

Peripheral nerve involvement is a frequent complication ofHuman Immunodeficiency Virus (HIV) infection1. Amongcranial nerves, the seventh is the most commonly affected2.However, facial diplegia is a rare clinical entity in the HIVinfected population. We recently observed a case that is ofparticular interest, since the patient developed facial diplegia andunilateral vestibular neuritis revealing HIV infection.CASE REPORTThe patient is a 60-year-old heterosexual male with a pastmedical history of morbid obesity and chronic obstructivepulmonary disease. He had been in his usual state of health untilapproximately four weeks prior to admission, when hedeveloped low grade fever, rhinorrhea, sore throat and diffusearthromyalgias. Fever resolved promptly after treatment withamoxycillin and clavulanic acid. Later on he was admitted to ourinstitution with a five-day history of constant dizziness,unsteadiness and imbalance. By the time of admission headditionally experienced perioral numbness followed suddenlyby lack of facial movements, with failure of both eyes to closeand difficulty in sucking, smiling and talking. Neurologicalexamination revealed bilateral peripheral facial palsy, right-deviation tendency in past pointing and Quix tests, and mildunsteadiness with gait deviation to the right. The remainder ofphysical and neurological examination was normal, includingintact reflexes. Laboratory studies disclosed an erythrocytesedimentation rate of 51 mm/h. White blood cell count was 12.4x 109 cells/l, with 35.6% neutrophils, 48.6% lymphocytes and12.3% monocytes. Red blood cell and platelet counts werewithin normal limits. Autoantibody screening tests and bloodbiochemistry parameters, including angiotensin convertingenzyme, were also normal. Cerebrospinal fluid (CSF) contained30 cells/µl with 93% lymphocytes, protein 1.64 g/l and glucose2.6 mmol/l (serum glucose 6.7 mmol/l). Cytology was negative.No organism was seen on Gram-stained smear, and cultures ofCSF for bacterial, mycobacterial, viral and fungal organismswere also negative. Polymerase chain reaction and serologies forcitomegalovirus, varicella zoster virus, Epstein-Barr virus, type1 and 2 herpes simplex virus, Toxoplasma gondii, Cryptococcus,Brucella, Treponema pallidum, and Borrelia burgdorferi werenegative in both serum and CSF. Human ImmunodeficiencyVirus testing was positive by ELISA and was confirmed byWestern blot. CD4+ cell count was 252 cells/µl and CD8+ cellcount was 5027 cells/µl, with CD4/CD8 ratio at 0.05. Viral RNAload was 197 000 copies/ml. A gadolinium enhanced brainmagnetic resonance imaging showed normal findings. Electro-physiological study confirmed severe impairment ofTHE CANADIAN JOURNAL OF NEUROLOGICAL SCIENCES 527Facial Diplegia and Vestibular Neuritis Secondaryto HIV Seroconversion SyndromeGállego Pérez-Larraya J., Riverol M.Can. J. Neurol. Sci. 2009; 36: 527-528BRIEF COMMUNICATIONStrigeminofacial reflex, with no motor evoked potentials recordedfrom the orbicularis oculi muscle of both sides after stimulatingthe supraorbital nerve. No evidence of a generalizeddemyelinating polyneuropathy was found on nerve conductionstudies. Since there was no evidence of any other underlyingsystemic illness, the diagnosis of right vestibular neuritis andfacial diplegia in relation to HIV acute seroconversion syndromewas made. After five days unsteadiness and facial palsy steadilyimproved, with complete resolution of neurological signs withinthe following week. At three-months follow-up the patientremained free of all neurological features.DISCUSSIONBilateral facial palsy, defined as the appearance of paresis ofthe contralateral facial nerve within 30 days of the onset of thefirst side3, is due to a systemic cause in a much higher incidencethan unilateral palsy. The diseases most commonly associatedwith facial diplegia are Guillain-Barre syndrome, multipleideopathic cranial neuropathies, brainstem encephalitis,Melkersson-Rosenthal syndrome, syphilis, sarcoidosis, Lymedisease, bacterial meningitis, poliomyelitis, Epstein-Barr Virusand other herpesvirus infections, leukemia, meningealcarcinomatosis, pontine tumours, Moëbius syndrome, ideopathicintracranial hypertension and, more rarely, HIV infection4,5.Although both unilateral and bilateral facial paralyses occur witha greater frequency in HIV infected patients than in the generalpopulation, bilateral palsy is exceedingly rare even amongthem5. Seventh nerve involvement is the most frequent cranialneuropathy associated with HIV2. As in our patient, it mainlyoccurs in the early stages of the disease, in the setting oflymphocytic pleocytosis and antibodies directed against HIV inCSF, supporting the hypothesis of a direct lesion of the facialnerve by the neurotropic virus5; however, an immunologicallymediated inflammation of the cranial nerves has also beensuggested as a possible pathophysiologic mechanism, given thesimilarities with other demyelinating peripheral neuropathiesassociated with HIV seroconversion6. Other causes of facialpalsy such as Ramsay-Hunt syndrome, syphilis, type-2 herpesFrom the Department of Neurology, Clínica Universitaria de Navarra, University ofNavarra, Pamplona, Spain.RECEIVED JANUARY 19, 2009. FINAL REVISIONS SUBMITTED MARCH 12, 2009.Correspondence to: J. Gállego Pérez-Larraya, Department of Neurology, ClínicaUniversitaria de Navarra, University of Navarra, Pío XII 36, 31008 Pamplona, Spain.simplex infection, mononeuritis multiplex, and lymphomatous orsarcomatous infiltration of the nerve, must be considered in thelater stages6,7. Our case is original in that facial palsy appearedand resolved simultaneously in both sides, in a completesymmetrical way. Moreover, it’s association to vestibular neuritisrevealing HIV infection is a very rare clinical picture, whichcould have been easily misdiagnosed due to its autolimitedcourse. To the best of our knowledge, this is the first report ofsimultaneous facial diplegia and vestibular neuritis revealingHIV infection. This diagnosis could be made since the extensivesearch for other causes was negative, serological testing for HIVinfection was positive and all symptoms resolved spontaneously.Without a high index of suspicion, physicians may miss thediagnosis of acute HIV infection. Symptoms, if present, arenonspecific and resolve spontaneously without treatment.Moreover, patients generally have no findings on physicalexamination except for possible lymphadenopathy. This reportemphasises that cranial nerve involvement such as facial palsy orvestibular neuritis can be the first symptom of HIV infection.Therefore HIV should be considered in the investigation of theseentities, more concretely facial diplegia, especially in patientswith high-risk behaviours even in absence of the mononucleosis-like syndrome. Given that patients are typically highly infectiousduring acute HIV due to a very high viral load8, the identificationof these patients, who are unaware of their seropositive status, isclearly important from a public health perspective.REFERENCES1. Parry GJ. Peripheral neuropathies associated with humanimmunodeficiency virus infection. Ann Neurol. 1988;23 Suppl:S49-53.2. Casanova-Sotolongo P, Casanova-Carrillo P, Casanova-Carrillo C.Diseases of the peripheral and visual nervous system duringinfection with human immunodeficiency virus. Rev Neurol.2003;37:481-5.3. Gevers G, Lemkens P. Bilateral simultaneous facial paralysis:differential diagnosis and treatment options. A case report andreview of literature. Acta Otorhinolaryngol Belg. 2003;57:139-46.4. Keane JR. Bilateral seventh nerve palsy: analysis of 43 cases andreview of the literature. Neurology. 1994;44:1198-202.5. Serrano P, Hernandez N, Arroyo JA, de Llobet JM, Domingo P.Bilateral Bell palsy and acute HIV type 1 infection: report of 2cases and review. Clin Infect Dis. 2007;44:57-61.6. Abboud O, Saliba I. Isolated bilateral facial paralysis revealingAIDS: a unique presentation. Laryngoscope. 2008;118:580-4.7. Kohler A, Burkhard P, Magistris MR, Chofflon M. Isolatedperipheral facial paralysis and HIV infection: 7 cases. RevNeurol. 1995;151:332-7.8. Daar ES, Moudgil T, Meyer RD, Ho DD. Transient high levels ofviremia in patients with primary human immunodeficiency virustype 1 infection. N Engl J Med. 1991;324:961-4.THE CANADIAN JOURNAL OF NEUROLOGICAL SCIENCES528

Facial diplegia and vestibular neuritis secondary to HIV seroconversion syndrome

J. Gállego Pérez-Larraya

M. Riverol

Crossref

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques