MOC September 2014: Twitchy Young Man

20 year old male presents to the ED with sudden facial twitches, as well as episodes of shaking and twitching lasting about one minute. He answers questions in one word sentences. One week ago, he was seen in another ED and was diagnosed with “syncope or seizure”. He had imaging and blood work there which was normal.

Social: Clean cut, A+ student at a college. Here with family
Meds: None
Med Hx: None
Allergies: None

Physical Exam
BP – 145/80
HR – 84
RR – 16
Temp – 98.4

Gen: NAD, answers in one word sentences, “odd”
HEENT: Normal OP, Normal TM bilaterally
Neck: Supple
CV: RRR
Resp: CTA B
Abd: Non-tender, No masses, No HSM
Extremties: moves all extremities normal, No rashes
Skin: No rashes
Neuro: One word answers, CN II-XII intact, motor 5/5 throughout, Negative rhomberg, Normal finger-nose-ringer, No pronator drift
Psych: Denies depression, SI, HI

What is on the differential diagnosis?
Tox, Seizure, Psych, Encephalitis, CVA, electrolytes, dehydration

Where do we start?
- IV, O2, monitor –- done
- ABC’s fine
- Labs/imaging – Pretty broad net

• CBC, BMP, LFTs?, Lactate?, ECG?, CT head vs MRI, TSH, Tox screen (Tylenol/ASA and Urine drug screen)
• All of this is completely normal (imagine no results in red on the screen)

What next?
No answer with tests above, but obviously something is going on. Is this mental health? Psych Evaluation? Lumbar Puncture?

• LP shows 7 WBC (all lymphocytes), Very mildly elevated Protein and normal glucose

After examination, the patient starts to have increasing episodes of the “twitching”. He then has what appears to be a full seizure.
- What now? Watch it happen? Meds?
- Ativan x 2 and loaded with Dilantin
- Still seizing -- Intubation and Propofol infusion, Keppra

Sent to the ICU...and we never hear from him again...
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but let's talk about...

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Anti-NMDA Receptor Encephalitis


- Name is pretty self-explanatory

• This receptor in the brain in cortex that is involved in synaptic transmission

- Many patients present with prodromal headache, fever, or a viral-like process, followed in a a viral-like process, followed in a few days by a multistage progression of symptoms that include:

• Prominent psychiatric manifestations (anxiety, agitation, bizarre behavior, hallucinations, delusions, disorganized thinking); isolated psychiatric episodes may rarely occur at initial onset or at relapse [56]
• Insomnia
• Memory deficits
• Seizures (75%)
• Decreased level of consciousness, stupor with catatonic features
• Frequent dyskinesias: orofacial, choreoathetoid movements, dystonia, rigidity, opisthotonic postures (85% with lip smacking)
• Autonomic instability: hyperthermia, fluctuations of blood pressure, tachycardia, bradycardia, cardiac pauses, and sometimes hypoventilation requiring mechanical ventilation.
• Language dysfunction: diminished language output, mutism. Echolalia is often noted in the early stages or in the recovery phase of the disorder.

- Findings

• CSF lymphocytic pleocytosis or oligoclonal bands (60%) (although CSF can be normal initially).
• EEG with infrequent epileptic activity, but frequent slow, disorganized activity that 

-Disease of the young

• Mean age 21
• Only 5% over the age of 45, 37% under age 18
• 38% have a paraneoplastic syndrome does not correlate with most abnormal movements.
• If it is, it is in women 
• 94% of which are ovarian teratomas
• 50% of women over the age of 18 are diagnosed with an Ovarian Teratoma

-This represents up to 4% of encephalitis, and up to 20% of cases for patients under the age of 30

• MUCH MORE COMMON THAN ALL OTHER KINDS OF ENCEPHALOPATHIES, INCLUDES 

- Tests: Anti-NMDA receptor antibodies

- Treatment:

• IVIG
• Methylprednisone 
• 5 days

- So, is this potentially worth starting treatment in the ED? At least discussion with intensivist/neurologist for discussion... and at least add on the lab

- Remember -- No necessary WBC count, fever,

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Questions
Which type of tumor is most likely in a patient with Anti-NMDA Receptor Encephalitis?
1. Hodgkin’s lymphoma
2. Neuroblastoma
3. Ovarian teratoma
4. Small cell carcinoma

Which of the following findings can be expected on lumbar puncture?
1. Decreased glucose
2. Decreased protein
3. Increased number of erythrocytes
4. Oligoclonal bands

True/False:
Most patient’s with this disease will have a high fever or an elevated WBC count.

True/False:
Anti-NMDA receptor encephalitis is thought to be the most common cause of encephalitis in younger patients.

Which of the following is the treatment of choice for anti-NMDA receptor encephalitis?
A. Acyclovir
B. Plasmaphoresis
C. IVIG
D. Methylprednisone
E. All of the above
F. None of the above
G. A and B only
H. A, B and C only
I. A and C only
J. A, B and D only
K. A, C and D only
L. B and D only
M. A and D only
N. C and D only