1. 1st Case Conference of the Year Sheryl Kho, M.D. PGY 3 July 22, 2009

2. Chief Complaint 17 yo AA boy “I can’t move my face.”

3. History of Present Illness 6 days PTA- Woke up unable to move R side of face No fever, rash, HA, constitutional sxs, trauma Went to ED- Dx: Bell’s Palsy Rx: Acyclovir + Prednisone

4. History of Present Illness 3 days PTA- persistent facial paralysis with pins and needle sensation + HA-frontal, +photophobia, +phonophobia +vomiting +pain in R ear +hyperacusis +tingling sensation on his tongue

5. Past Surgical Hx S/p I&D Pilonidal abscess- 2 wks ago Rx: Augmentin x 7 days

6. Past Medical Hx Varicella @ 5yo Occasional cold sores on upper lip IUTD PPD negative- 1 year ago

7. Adolescent Hx (HEADSSS) Lived in the Bronx No travel Denies tick bites, animal exposure Junior in HS, worked as a lifeguard during summer Denies sexual activity Denies use of illicit drugs or alcohol

8. Physical Exam VS: T 38.2C, HR 110bpm, RR 20/min, BP 127/75, SaO2 100% AAO, c/o frontal HA HEENT: NCAT, PERRLA, +crusted lesion in R ear canal, TM intact B/L, +2 crusted sores on R upper lip, MMM, clear OP, supple neck, no Brudzinski, no Kernig,+nuchal rigidity Lungs: CTA B/L, no WRR, no retractions Heart: RRR, normal S1/S2, no mrg

9. Physical Exam Abd: +BS, soft, NT, ND, no HSM Ext: FROMx4, no cyanosis, no edema, 2+pulses, good cap refill Neuro: AAO, unable to close R eye, +drooping R side of face with flattening of ipsilateral nasolabial fold, unable to wrinkle R side of forehead, unable to puff out R cheek, +asymmetric smile

10. Differential Diagnosis? Infectious  AOM, COM, cholesteatoma  Meningitis  Bell’s Palsy  Lyme Disease  Viral Syndrome  Mumps  Herpes zoster oticus Neuro/CNS  Mass/Tumor ie. Acoustic neuroma, glomus tumor,facial ner neuroma  Stroke  Bleed Others  Vertigo  Trigeminal Neuralgia  TMJ Disorders  Dental Pain  Persistent Idiopathic Facial Pain Toxins  Tetanus Iatrogenic  Surgical  Embolization  Nerve block Idiopathic  Autoimmune syndrome  Myasthenia gravis  Multiple sclerosis  Sarcoidosis  Amyloidosis Systemic  DM  Alcoholic neuropathy  Hyperthyroidism  Pregnancy Trauma  Birth trauma  Temporal bone fracture  Facial trauma

11. Diagnostic Workup? CBC, BCx CMP CSF, CSF Cx Lyme titers CT scan Wound Cx

12. Diagnostic Workup CBC- 4.4>13<172,000 N65 L19 M16 CMP- normal TP: 7.5 Alb: 4 UA-normal CT Brain- negative Lyme titers negative IgM, IgG

13. Diagnostic Workup Lumbar Puncture  Clear CSF  WBC: 32, L91, RBC: 25  CSF protein 45, CSF glucose 47  Gm stain: no organisms, no cells  CSF culture pending  CSF PCR HSV negative  CSF Viral Cx negative

14. Management Started on Ceftriaxone and Acyclovir Prednisone taper HA and neck stiffness resolved w/in 24hr CTX d/c’d once CSF cx negative

15. Further Diagnostic Workup VZV cultured from R ear canal lesions HIV ELISA: positive CD4 count: 28  AIDS HIV Viral load: 414,555

16. Further Management IV Acyclovir continued Prednisone PO x 7 days Bactrim and Zithromax- prophylaxis for M. avium, Pneumocystis jiroveci Efavirenz, Emtricitabine and Tenofovir started 6 wks after acute illness

17. What happened later? 6 months after start HAART  Viral load: undetectable  CD4 count: 220  Continues with sensitivity to sound and R facial paralysis

18. Ramsay Hunt Syndrome

19. 1907: described by James Ramsay Hunt “Geniculate neuralgia”, “nervus intermedius neuralgia”  Facial paralysis  Inner ear dysfunction  Periauricular pain  Herpetiform vesicles of the pinna (herpes zoster oticus)

20. Ramsay Hunt Syndrome Primary infection with VZV (HHV 3) Latent in the geniculate ganglion of CN VII VZV reactivation, zoster: decline in cell mediated immunity ie. HIV

21. Pathophysiology of RHS Geniculate ganglion of CN VII Petrous portion of the temporal bone lies the ear apparatus (inner ear) CNVII courses through the inner and middle ear Inflammation causes facial paresis, vertigo, otalgia, hyperacusis

22. Anatomy of the Facial Nerve

25. Epidemiology of RHS Rare Complete recovery rate <50% Self limiting Morbidity: facial weakness

26. History Taking Pain deep in the ear  Vertigo  Tinnitus  Facial paresis Rash, blisters, herpetic lesions

27. Physical Examination Pain Peripheral facial nerve paralysis with herpetic lesions  Ant 2/3 of tongue  Soft palate  ext auditory canal  Pinna Ipsilateral hearing loss, balance problems Neuro exam

28. Physical Examination

29. Diagnostic Workup CBC with differential ESR Serum electrolytes Viral Studies  Serologic tests  VZV PCR on tear samples  Viral cxs Imaging studies  MRI, CT scan Audiometry CSF studies (controversial)

30. RHS in HIV Patients Normal children: 0.74/1000 >70% in HIV, CA 7-20x greater risk than children with leukemia Recurrence: 53% (1.7-5%) Persistence of skin lesions: 14%

31. Bell’s Palsy  Idiopathic facial paralysis (IFP)  Virally mediated, exact mechanism unknown  Affects CN VII  Reactivation of HSV  60-75% of acute facial palsies  Sudden paresis of facial muscles on one side, absence of CNS dse <48hrs  20-30 pxs/100,000  Paresis in the morning, worsens thru the day Otalgia, facial pain, hyperacusis, decreased tears, NO SKIN LESIONS

32. Herpes Zoster Ophthalmicus Primary infection: chickenpox Latent in the trigeminal ganglion Affects the first division of CN V PE:

33. Treatment of RHS Acyclovir + prednisone Remains controversial

34. Thank you…