Today in Goldstein Morning Report we presented a case of a 45 year old M with no PMH who was brought in for acute encephalopathy and witnessed seizures. On exam the patient was found to have multiple cranial neuropathies and labs were significant for a positive RPR (1:32). The patient had a lumbar puncture performed which showed 4 WBCs (lymphocytes), glucose 67 and protein 54 and CSF VDRL was found to be reactive.

Neurosyphilis
– Clinical signs of neurosyphilis include meningitis, stroke, acute/chronic encephalopathy, loss of vibration sense, cranial nerve dysfunction and auditory/ophthalmic abnormalities.
– The diagnosis of neurosyphilis is based on clinical suspicion, serologic tests and CSF analysis.
– Remember that CSF abnormalities can occur in early syphilis, and may not be clinically significant in the absence of neurologic signs/symptoms.
– In early neurosyphilis, serum nontreponemal (VDRL, RPR) and treponemal tests (FTA-ABS) are generally reactive, however in late neurosyphilis VDRL and RPR may be nonreactive and FTA-ABS should always be performed.
– CSF-VDRL is highly specific and a reactive test is diagnostic for neurosyphilis, however the sensitivity is much lower.

Diagnosis of neurosyphilis in an HIV- patient
– In patients with a nonreactive CSF-VDRL, treatment depends on further CSF findings.
—> If the CSF WBC>5, treat for neurosyphilis.
—> If the CSF WBC<5 but the CSF protein >45, treat for neurosyphilis.

Diagnosis of neurosyphilis in an HIV+ patient
– Similarly to HIV- patients, further CSF studies must be used to diagnosed neurosyphilis when the CSF-VDRL is negative. However HIV can cause a mild CSF pleocytosis and protein level (especially in those with CD4>200, detectable HIV RNA and not on ART).
–> If the CSF WBC >20, treat for neurosyphilis.
–> If the CSF WBC is 6-20, treat for neurosyphilis if the CD4<200, HIV RNA<50 and on ART
–> If the CSF WBC is ≤5, do not treat.

Today we presented a case of a 35 year old female with no past medical history presenting with 3 weeks of fevers, rash, sore throat, weight loss and diffuse joint pain. The differential for this presentation is broad, including infection, malignancy and rheumatologic disorders. Labs were significant for very mildly elevated transaminases, mild hyponatremia and normal white count with elevated neutrophils.

– Given the constellation of symptoms, multiple infectious causes were considered including murine typhus, acute HIV, and parvovirus (the rash associated with parvovirus can intensify with warm water and showers). The patient reported eating queso fresco so Brucellosis and Q fever were also possible (chronic Q fever can present with high fevers and mild transaminitis).
– Malignancy was considered, in particular hematologic malignancy given the presence of B symptoms. However the presence of joint pain and evidence of synovitis made hematologic malignancies less likely. Rheumatologic diseases such as RA and SLE can also present with similar symptoms.

The patient was ultimately diagnosed with Adult Onset Still’s Disease after infection and other rheumatologic diseases were ruled out, and iron panel showed a ferritin of >8000. Still’s disease is a diagnosis of exclusion made upon characteristic clinical and laboratory features.
– Our patient satisfied 3/4 of the major Yamaguchi criteria (patient did not have leukocytosis >10,000 however did have a PMN predominance) and 3 minor criteria (listed below).

Thanks to PGY3 Alex Yang for doing a great presentation!

Today’s morning report case involved a 34 year old male with an unknown psychiatric history who was brought in after being found altered, with left sided drooling and increased tone in all extremities. Vital signs showed hypertension and tachycardia and labs were significant for elevated liver enzymes (AST>>ALT), mild AKI and a CK level of 30,618. Patient was diagnosed with neuroleptic malignant syndrome (NMS) after it was discovered that he had been taking Haldol, Risperdone and Sertraline.

Take home points from today’s case:

• NMS most commonly occurs with use of 1st generation antipsychotics but can also occur with 2nd generation (clozapine, risperidone) and certain antiemetics (metoclopramide, prochlorperazine)
  • Remember that the abrupt discontinuation or dose reduction of Parkinson medications (dopaminergic agents such as levodopa) can also cause NMS!
• NMS generally occurs after weeks of therapy, but can occur as early as after the 1st dose or after several years.
• The mnemonic FEVER (from Step 1) can help identify NMS: Fever, Encephalopathy, Vital sign instability (tachycardia, labile BP), Enzyme elevation (CK), Rigidity (lead-pipe)
• The main differential diagnoses for NMS include serotonin syndrome (see below table) and malignant hyperthermia (occurs after inhalational anesthetic agents and succinylcholine)
• Prompt recognition and discontinuation of the causal agent is important, followed by aggressive supportive care and benzodiazepines for agitation.
  • In severe cases, pharmacologic agents such as dantrolene/bromocriptine can be used, however these have not been studied in large trials and are based upon case reports.
• Oral antipsychotics can be slowly reintroduced and slowly titrated after 2 weeks.