Id case conference october 31 2007
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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, 4 th , and 5 th digits

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ID Case Conference October 31, 2007

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Id case conference october 31 2007

ID Case Conference October 31, 2007

Meredith Niess

MS3

UNC-SOM


Id case conference october 31 2007

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.


Id case conference october 31 2007

HPI

  • ED visit 7/28 – around time of onset of symptoms.

    • 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

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.


Id case conference october 31 2007

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


Id case conference october 31 2007

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.


Id case conference october 31 2007

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.


Id case conference october 31 2007

PMH

  • Allergies:

    • Keflex causes diarrhea

  • Medications:

    • 81 mg Ecotrin

    • Vitamin C


Review of systems

As mentioned in HPI

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

Review of Systems


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

Physical Exam


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

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

Neuro Exam


Labs 10 17

13.0

292

39.0

Labs – 10/17

Procedures Ordered

ESR: 88

Hgb A1C: 5.6

CO2: 25

BUN: 8.0

Creat: 0.8

GFR >60

BUN/Cr: 10

AST: 23

ALT: 29

B12: 412

Folate: 6.2

  • MRI

  • MRA – Circle of Willis

  • Carotid Duplex

  • Labs shown plus baseline cholesterol

4.2

MCV: 81

No diff


Labs 10 19 ed

12.1

255

36.6

Labs – 10/19 (ED)

CSF:

TNC:67

RBCs: 11

% PMNs: 2

% Lymphos: 88

Protein: 117 (15-45)

Glucose:49 (50-75)

BMP – wnl

5.2

Urine tox screen negative

Hepatitis panel negative

UA and culture wnl


Id case conference october 31 2007

8/6 ED visit

Negative CT

D/C with symptoms attributed to dementia


Discussion

Discussion


Id case conference october 31 2007

  • “…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

Diagnostic Procedure Performed

  • RPR+ at 1:>16,384

  • HIV ELISA positive

  • VDRL of CSF was positive at 1:8


Neurosyphilis

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

Natural History of Neurosyphilis

  • See UpToDate (on campus only)


Id case conference october 31 2007

  • Early Neurosyphilis

  • Affects CSF, meninges, and vasculature

  • Months to years after infection

  • asymptomatic – dx based on CSF

  • symptomatic

    • Meningitis – usually 1st year

    • Meningovascular – often presents with ischemic stroke

  • Late Neurosyphilis

  • Affects brain and spinal cord parenchyma

  • 10+ years after infection

  • General paresis – progressive dementing illness (10-25 years)

  • Tabes dorsalis


Clinical features symptoms of neurosyphilis

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

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

  • 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

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


Diagnosis1

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

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

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

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

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

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.


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  • Neurosyphilis

    • Case Reports

    • Reviews

    • Differential Diagnosis

    • Drug Therapy


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