Ramsay Hunt syndrome in focal segmental glomerulosclerosis.

acial paralysis has been reported in the literature subsequent to multiple insults but its occurrence after herpes zoster infection is a drastic, disfiguring and painful event with poorer outcomes. Ramsay Hunt syndrome (RHS) is characterized by the presence of cochleovestibular symptoms in association with facial paralysis. Facial nerve palsy has long been considered to have mainly infectious etiology. Recent diagnostic analysis have provided convincing evidence that the virus most likely enters the geniculate ganglion during chicken pox via sensory branches of facial nerve and remains in dormancy before it is reactivated. This case presents the lower motor neuron facial paralysis along with vesicular rashes in the external auditory canal and pinna, giddiness, otalgia and hearing impairment in a young patient diagnosed histopathologically as having focal segmental glomerulosclerosis subsequent to nephrotic syndrome, 3 months before. Our patient is a 14-year-old Saudi girl who reported in the emergency room with the complaints of sore throat, cough, fever and some dyspnea for 4 days. A probe into past history revealed that the girl had nephrotic syndrome along with hypertension diagnosed based on clinicals and investigations 3 months before and was put on steroids and anti hypertensives. One month before renal biopsy at the King Faisal Specialist Hospital, Riyadh, Kingdom of Saudi Arabia was carried out and a diagnosis of focal segmental glomerulosclerosis was established and the patient was advised to use nifedipine, furosemide and to continue oral steroids. Patient’s parents could not exclude the occurrence of chickenpox in her childhood. On general examination at the time of recent presentation, her temperature was 38.7°C, blood pressure 140/100 mm Hg, pulse rate of 120 per minute regularly and respiratory rate was 28 per minute. Her throat examination revealed hyperemic posterior pharyngeal wall and tonsils with jugulodigastric lymphadenopathy. The patient was admitted for the management of acute upper respiratory tract infection and was administered parenteral cefuroxime. Approximately 18 hours after admission, the patient developed skin eruptions on lateral surface of right auricle (Figure 1 ) along with severe otalgia. Eruptions were vesicular in nature, hyperemic and tender. There was a small rash on the right cheek. The pain was experienced in the right ear and para auricular areas. Approximately 24 hours after admission the patient complained of facial asymmetry of the angle of mouth. Patient had developed right sided lower motor neuron type facial paralysis. The next day, the patient felt more pain and slight numbness on the right side of the face. The vesicles became prominent and extended to the external auditory canal although drumhead was spared but hyperemic. She also had off and on dizziness spells and some difficult in hearing on right side. She was examined vigilantly at regular intervals and a battery of investigation was carried out. Blood routine examination showed that hemoglobin was 12.5g/L, white blood cell was 12.6 and the differentials within the normal limits. Urine routine examination showed turbid appearance, red blood cells 40-45, albumin 2++ and increased concentration of urobilinogen. Urine culture grew E coli, resistant to cephradine, co-trimoxazole, genticyn and ampicillin but sensitive to nitrofurantoin and nalidixic acid. Urea and creatinine were within normal. Uric acid was raised up to 325, while sodium and potassium were at lower normal limits 132 and 3.5. Liver and lipid profiles and other biochemical analysis had unremarkable changes. Skin scrapping from the vesicles was also subjected to culture but no bacterial growth was reported. Ultrasonic renal imaging revealed multiple echopenic spots suggestive of infarcts. Schermir test showed reduced lacrimation on the effected side. Pure tone audiometry results were in favor of mild sensorineural hearing loss. The facilities such as electrical stimulation tests, magnetic resonance imaging, viral studies, polymerase chain reaction (PCR) and other sophisticated audiological and serological tests and so forth, which have been used as diagnostic tools in such cases are not available at our center. Based on the clinical information and investigations, the diagnosis of RHS was established in this patient who has focal segmental glomerulosclerosis and has immunosuppressed status due to steroids intake. She