Transcript WELCOME! 1st Case Conference of the Year

1st Case Conference of the
Year
Sheryl Kho, M.D.
PGY 3
July 22, 2009
Chief Complaint
17 yo AA boy
“I can’t move my face.”
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
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
Past Surgical Hx
S/p I&D Pilonidal abscess- 2 wks ago
Rx: Augmentin x 7 days
Past Medical Hx
Varicella @ 5yo
Occasional cold sores on upper lip
IUTD
PPD negative- 1 year ago
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
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
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
Differential Diagnosis?
 Infectious
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AOM, COM, cholesteatoma
Meningitis
Bell’s Palsy
Lyme Disease
Viral Syndrome
Mumps
Herpes zoster oticus
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Toxins
 Tetanus
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Iatrogenic
 Surgical
 Embolization
 Nerve block
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Idiopathic
 Autoimmune syndrome
 Myasthenia gravis
 Multiple sclerosis
 Sarcoidosis
 Amyloidosis
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Systemic
 DM
 Alcoholic neuropathy
 Hyperthyroidism
 Pregnancy
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Trauma
 Birth trauma
 Temporal bone fracture
 Facial trauma
 Neuro/CNS
 Mass/Tumor ie. Acoustic neuroma,
glomus tumor,facial ner neuroma
 Stroke
 Bleed
 Others
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Vertigo
Trigeminal Neuralgia
TMJ Disorders
Dental Pain
Persistent Idiopathic Facial Pain
Diagnostic Workup?
CBC, BCx
CMP
CSF, CSF Cx
Lyme titers
CT scan
Wound Cx
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
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
Management
Started on Ceftriaxone and Acyclovir
Prednisone taper
HA and neck stiffness resolved w/in 24hr
CTX d/c’d once CSF cx negative
Further Diagnostic Workup
VZV cultured from R ear canal lesions
HIV ELISA: positive
CD4 count: 28  AIDS
HIV Viral load: 414,555
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
What happened later?
6 months after start HAART
Viral load: undetectable
CD4 count: 220
Continues with sensitivity to sound and R facial
paralysis
Ramsay Hunt Syndrome
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)
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
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
Anatomy of the Facial Nerve
Anatomy of the Facial Nerve
Anatomy of the Facial Nerve
Epidemiology of RHS
Rare
Complete recovery rate <50%
Self limiting
Morbidity: facial weakness
History Taking
Pain deep in the ear
Vertigo
Tinnitus
Facial paresis
Rash, blisters, herpetic lesions
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
Physical Examination
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)
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%
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
Herpes Zoster Ophthalmicus
 Primary infection: chickenpox
 Latent in the trigeminal ganglion
 Affects the first division of CN V
 PE:
Treatment of RHS
Acyclovir + prednisone
Remains controversial
Thank you…