1st Case Conference of the Year

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,+nuchalrigidity • 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? • 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 • 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

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. Ramsay Hunt Syndrome • 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

23. Anatomy of the Facial Nerve

24. 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…