Transcript Document

ID Case Conference
October 31, 2007
Meredith Niess
MS3
UNC-SOM
HPI
CC – “right-sided weakness”
• 50 y/o right-handed man c/o of approximately 6 week
history of right leg, hand, and facial weakness.
• Reports numbness of right palm, 4th, and 5th digits
• Reports difficulty with slower slurred speech
• Memory impairment, having lost 2 cell phones in 1
month.
HPI
• ED visit 7/28 – around time of onset of symptoms.
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c/o Blurred vision, memory deficit, dizziness, unsteady gait.
Normal PE, including neuro exam with nl gait
Glucose finger stick – 178 mg/dL
Symptoms attributed to uncontrolled diabetes
• ED visit 8/6 – similar, slightly exacerbated presentation
– c/o blurry vision, “shaky”, speech slurring, right- sided weakness,
gait difficulties, memory problems.
– PE positive for mild aphasia, “sluggish” pupillary exam, and mild
cerebellar findings. Otherwise negative.
– CBC, BMP and UA all WNL
– Head CT done, normal.
– Discharged from ED with diagnosis of new onset dementia,
referred to neuro.
HPI continued
• Patient was seen in neurology, diagnosed with
new onset right-sided hemiparesis – ataxic
presentation. They were suspicious of lacunar
strokes given his diabetic history.
• Stroke work-up initiated in Neuro Clinic
• Patient presented to ED two days after neuro
appointment with persistent symptoms – 6
weeks after initial onset of symptoms.
PMH
• Medical:
– Diabetes Mellitus type 2 – dx 1999.
• d/c medical management 2/2 to cost.
• No history of retinopathy or nephropathy
– Otherwise unremarkable
• Surgical: none reported
PMH
• Social hx:
– Smoking: 1 pack/week until quit 6 weeks ago
– EtOH: 6 drinks/week until quit 3-6 months ago, denies prior
problems with addiction.
– Drugs: Denies IV or other drug use
– Sex: Denies being sexually active for 4-5 years. States previous
sexual activity restricted to female only monogomous
relationships. No history of STDs. Single.
– Occupation: Cook at UNC sorority until 6 weeks previous when
symptoms prevented him from working
– Residence: Lives with 2 long-term friends
– Family: Mom and sister live in Maryland but came in town to help
him.
– Pets: No pets
– Travel: No travel outside east coast.
PMH
• Family hx:
– Father – deceased at 85: complication related
to dementia.
– Mother, brother, and sister alive and healthy.
– No family history of early onset dementia or
other neurologic conditions.
PMH
• Allergies:
– Keflex causes diarrhea
• Medications:
– 81 mg Ecotrin
– Vitamin C
Review of Systems
• As mentioned in HPI
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Vision changes
Balance/gait difficulty
Right-side facial droop
Right-sided weakness
Right-sided neuropathy
Memory deficit
aphasia
• 20-30 lb weight loss over
2-3 months
• Excessive fatigue
• Right knee joint pain
• Denies CP, SOB, HA,
fevers/chills/sweats, LE
swelling, n/v/d, or any
mood changes.
• All other systems
negative
Physical Exam
• Gen: well-appearing AA
man, NAD, mildly
overweight
• T: 36.4
HR: 70s
RR: 20
BP 140/70
Sat 98% RA
• HEENT: NAT, PERRL,
EOM+, MMM
• Neck: full ROM, no bruits,
neg for meningismus,
lymphadenopathy,
thyromegaly.
• Pulm: CTAB
• CV: RRR, nl S1, S2, no
m/r/g, pulses 2+ b/l in all
extremities
• Abdomen: obese, soft,
NT, +BS, no
organomegaly
• Skin: no rashes/lesion
• GU: no d/c, no lesions
• Extremities: no c/c/e, no
joint swelling
Neuro Exam
• Mental Status:
– A&O x 3
– Short term memory intact
– Attention and concentration
appropriate
– Adequate spontaneous speech
– Intermittent mild slurring of
speech
– Periodic hesitancy with speech
• Neurologic
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CN II-XII intact
Muscle tone WNL
Pronator drift: RUE
RLE weak:4/5; RUE weak: 4+/5
Cerebellar abnlities:
Dysdiadochokinesis, heel-to-shin
abnormality (unable to perform)
on RLE, finger-to-nose impaired
on RUE
– Normal gait
– Romberg Negative
– Position sense, and vibration
intact upper and lower ext. slight
difference b/w RLE and LLE on
pinprick
Labs – 10/17
Procedures Ordered
B12: 412
Folate: 6.2
4.2
13.0
39.0
MCV: 81
No diff
292
ESR: 88
Hgb A1C: 5.6
CO2: 25
BUN: 8.0
Creat: 0.8
GFR >60
BUN/Cr: 10
AST: 23
ALT: 29
• MRI
• MRA – Circle of Willis
• Carotid Duplex
• Labs shown plus
baseline cholesterol
Labs – 10/19 (ED)
BMP – wnl
12.1
5.2
36.6
255
CSF:
TNC:67
RBCs: 11
% PMNs: 2
% Lymphos: 88
Protein: 117 (15-45)
Glucose:49 (50-75)
Urine tox screen negative
Hepatitis panel negative
UA and culture wnl
8/6 ED visit
Negative
CT
D/C with
symptoms
attributed to
dementia
Discussion
• “…scattered nonspecific small areas of increased T2/FLAIR signal
in the subcortical white matter of the frontal lobes, left centrum
semi ovale, and left atria periventricular region. May represent the
sequela of chronic small vessel disease. No sequela of acute or
remote lacunar stroke is seen.”
• MRA wnl
• No evidence fluid collections, hemorrhage, or infarct
Diagnostic Procedure Performed
• RPR+ at 1:>16,384
• HIV ELISA positive
• VDRL of CSF was positive at 1:8
Neurosyphilis
• CNS infection by spirochete: Treponema
pallidum
• Occurs at any time after initial infection
• Epidemiology:
– Usually sexually transmitted
– Prior to abx occurred in 25-35% of people
with syphilis
– 1/3 asymptomatic, 1/3 tabes dorsalis, 10%
paresis
Natural History of Neurosyphilis
• See UpToDate (on campus only)
Early Neurosyphilis
• Affects CSF, meninges, and
Late Neurosyphilis
• Affects brain and spinal cord
vasculature
parenchyma
• Months to years after infection
• 10+ years after infection
• asymptomatic – dx based on CSF
• General paresis – progressive
dementing illness (10-25 years)
• symptomatic
- Meningitis – usually 1st year
- Meningovascular – often
presents with ischemic stroke
• Tabes dorsalis
Clinical Features - Symptoms of
neurosyphilis
• Personality change (including cognitive and/or behavioral
impairment) - 33%
• Ataxia - 28%
• Stroke - 23%
• Ophthalmic symptoms (eg, blurred vision, reduced color perception,
impaired acuity, visual dimming, photophobia) - 17%
• Urinary symptoms (eg, bladder incontinence) - 17%
• Lightning pains (larynx, abdomen, various organs) - 10%
• Headache - 10%
• Dizziness - 10%
• Hearing loss - 10%
• Seizures - 7%
From Timmermans M, Carr J. Neurosyphilis in the modern era.
J Neurol Neurosurg Psychiatry. 2004 Dec;75(12):1727-30.
Clinical Features - Signs of
Neurosyphilis
• Hyporeflexia - 50%
• Sensory impairment (eg, decreased proprioception, loss
of vibratory sense) - 48%
• Pupillary changes (anisocoria, Argyll Robertson pupils) 43%
• Cranial neuropathy - 36%
• Dementia, mania, or paranoia - 35%
• Romberg sign - 24%
• Charcot joint - 13%
• Hypotonia - 10%
• Optic atrophy 7%
From Timmermans M, Carr J. Neurosyphilis in the modern era.
J Neurol Neurosurg Psychiatry. 2004 Dec;75(12):1727-30.
Diagnosis
• Diagnosis based on clinical suspicion and
supportive laboratory values
– neurologic signs, +serum RPR
• Spinal Fluid Examination is key
– LP should be considered in any patient with
neurologic or ocular disease and history of
syphilis or unknown syphilis history.
– Suggestive findings include:
• CSF WBC > 5 lymphos/microL
• Protein > 45 mg/dL
Who Gets an LP?
• Neurologic or ophthalmic signs or symptoms in
any stage of syphilis
• Evidence of active tertiary syphilis affecting other
parts of the body
• Treatment failure in any stage of syphilis
• HIV with late latent syphilis or syphilis of
unknown duration
– Some experts recommend LP in all patients with
comorbid HIV and syphilis regardless of stage
• RPR titer>=1:32
Diagnosis
• Positive CSF VDRL is specific for diagnosis of
neurosyphilis
– Not sensitive – can be false negative in as many as
70% of patients with neurosyphilis
– Can be false positive if any blood is present in CSF
when sent to lab
• Positive CSF FTA-ABS is sensitive but not
specific.
• Difficult to diagnose if CSF-VDRL is nonreactive
Treatment
• Penicillin G 3-4 million units IV q4H for 10-14
days
• Note that standard late syphilis treatment is
benzathine penicillin 2.4 MU IM once per week
for 3 weeks.
• Suggested treatment for possibility of coexisting
latent infection is 3 additional doses of
benzathine penicillin.
– Based on pathophysiology of organism and safety of
drug.
– No clinical trial data available.
Treatment continued
• If patients have a severe PCN allergy they
should be desensitized
• F/U LPs performed 3-6 months after
treatment and every 6 months thereafter
until CSF WBC is normal and CSF-VDRL
is normal.
– If these are not met – retreat.
HIV and Syphilis
• Risk fx target similar populations
• Coinfection results in similar clinical
presentation at similar stage of disease
when compared to solitary syphilis,
however in HIV+ patients:
– Primary stage more often has multiple
chancres
– Secondary stage symptoms more likely to
present concurrent with primary chancre(s)
HIV and Neurosyphilis
• Recent review of 170 cases of suspected neurosyphilis
cases published in MMWR June 29, 2007
– From LA, NY, Chicago, San Diego between Jan 02-June 04.
– 67% had symptoms c/w early syphilis
– 58% were MSMs
• 86% of the MSM (49 patients) were reported to be HIV positive
• Of the 49 HIV-positive MSM with symptomatic early
neurosyphilis, 53% (26 patients) had no other signs or
symptoms of syphilis
• 30% had persistent symptoms at 6 months despite
documented serologic response. Persistent symptoms
were not associated with receipt of HAART, initial CD4,
initial viral load, or time from syphilis onset to treatment.
References
• UpToDate (on campus access only)
– Sparling, F. Late syphilis.
– Marra, C. Neurosyphilis.
– Rompalo, A. Syphilis and HIV infection.
• Timmermans M, Carr J. Neurosyphilis in the modern era. J Neurol
Neurosurg Psychiatry. 2004 Dec;75(12):1727-30.
• Centers for Disease Control and Prevention (CDC). Symptomatic
early neurosyphilis among HIV-positive men who have sex with
men--four cities, United States, January 2002-June 2004.
MMWR Morb Mortal Wkly Rep. 2007 Jun 29;56(25):625-8.
Search PubMed
• Neurosyphilis
– Case Reports
– Reviews
– Differential Diagnosis
– Drug Therapy