Coming in hotter than August, here is this month’s COtM presentation:
A 51 y/o M presents to your ED with 6 days of imbalance and difficulty walking. He says he feels that he cannot keep himself upright when he walks and that “nothing seems to line up” when he looks at an object. Prior to these symptoms the patient reports having a bout of abdominal cramping with bloody diarrhea for three days, diarrhea self-resolved. He visited an urgent care center two days ago where he had a CT head (which was negative) and was given meclizine for vertigo, which has not helped. Patient denies fevers, chills, weakness, numbness, dysphagia, nausea/vomiting, chest pain, or trouble breathing.
PMH: DM, HTN, HLD, CAD s/p stent 2 years ago
PSH: None
Meds: Metformin with long acting insulin, ACEI, Ca-channel blocker, statin, 81mg ASA
All: NKDA
ROS: As above
Vitals: 152/88, HR 92, Temp 98.0F, RR 15, 99% on RA, POC 198
PE: Negative aside from the following pertinent findings
Neuro – PERRLA, EOM reveals some weakness in eye movements with patient reporting double vision, no nystagmus, no ptosis, remainder of CN’s intact, motor 5/5 all muscle groups, sensation intact to light touch and pinprick in all areas, finger-to-nose intact, patient can maintain a seated position, reflexes decreased in all extremities, gait wide-based with truncal instability but normal movement of arms and legs, romberg (-).
So what do you think? Whoever answer the following questions with their best reasoning gets the glory and the win for this month:
1. What is your ranked differential diagnosis list?
2. What would your workup entail in the ED?
3. Is there any specific treatment for this patient? If so, what? If not, how is the disease managed?
The answer will be posted next week, so read up and start formulating your guesses!
– Hassel
James Hassel
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2 Comments
ablumenberg · August 8, 2015 at 1:30 am
To summarize the case, 51 year old man with DM, HTN, HLD, CAD with stent 2 years ago develops truncal ataxia, arreflexia and ophthamoplegia soon after some bloody diarrhea, presumably from c. jejuni.
The case is classic presentation of Miller-Fisher variant of Guillain-Barre syndrome. Work up would include LP to look for albuminocytologic dissociation, electromyelography, anti-Gq1b antibody and MRI of the spine and dorsal nerves. Just kidding! These are interesting to think about, but the patient has been sick for 6 days and what he NEEDS in the ED is a NIF (negative inspiratory force) and FVC (forced vital capacity) to assess respiratory muscle strength.
Consult neurology early, get the standard CBC, chem, coags (LP will occur at some point) but put them through phlebotomy because they are unlikely to change your management.
There are primarily two acutely life threatening aspects of AIDP (acute inflammatory demyelinating polyradiculopathy)
1. Paralysis of the respiratory muscles. The bedside pulmonary function tests will determine whether the patient should go to ICU or the floor – or be intubated. The patient can get the LP, EMG, MRI, etc. after admission. If the NIF is normal, do not be reassured. It should be repeated Q1 hour. About 1/3 of GBS patients require ICU admission. If the patient bedboards in the ED be aware that they can rapidly decompensate and require ventilator support. You should worry when the NIF is -30cm water and when the FVC is less than 15mL/kg.
2. Autonomic instability. Do not be reassured by “normal” vitals in a patient with GBS. Autonomic fibers are affected by the disease and the patient can develop dangerous swings in heart rate and blood pressure. The patient should be on a monitor, a real one.
Medical treatment is with IVIG which shortens symptoms by 50%, but this is probably best left to the inpatient team. Plasmapheresis has the same efficacy but more complications and is rarely used. Steroids have not been shown to be effective.
To summarize: for AIDP patients the ER management should focus on clinical diagnosis, management of respiratory function and hemodynamic stability, and decision whether to admit to floor or ICU. The crucial datapoints for disposition and management are H&P, NIF & FVC, vital sign TREND. Labs and imaging are secondary.
The broader differential diagnosis for this patient includes electrolyte disturbance, Wenicke-Korsakoff, hypothyroid, alcohol related cerebellar atrophy, paraneoplastic syndrome (hitting the cerebellum, especially the vermis), B12 defciency, multiple sclerosis, syphilis, heavy metal toxicity, inhalant toxicity, diabetes, cva, psychogenic, tick paralysis, Lyme disease, botulism.
edenkim · August 8, 2015 at 7:09 am
Ddx:
Guillan-barre (possibly miller-fisher variant)
cerebellar (likely midline) ataxia – from either hemorrhage or infarct
encephalitis
wernicke’s encephalopathy
MS
vertebrobasilar insufficiency
workup:
IV, O2, monitor
cbc, cmp, mg/phos, UA/UCx, ETOH level, shock panel
ekg, cxr
MRI head
LP to look for albuminocytologic dissociation, oligoclonal bands, cell count, gram stain, csf culture, protein
stool culture for c.jejuni
NIF
treatment:
IVIG or plasma exchange
steroids (+/- as evidence for its use is lacking, and may actually be harmful)
low threshold to intubate based on clinical picture and NIF
thiamine
neurology consult
ICU consult if patient has evidence of respiratory compromise
the preceding diarrhea could be a red herring, but could also be due to c.jejuni infection that is often associated with guillan-barre. also, given the other neurological symptoms (hyporeflexia, truncal ataxia, ophthalmoplegia), I’d go with miller fisher variant of GB as my top diagnosis as it does not appear to be an ascending paralysis but rather a descending. patient had a negative CT already so MRI may performed to better visualize pathology in the posterior fossa, and to look for evidence of MS. the LP could be used to better elucidate encephalitis vs. GB vs. MS based on cell counts/culture/etc. consults will help dispo the patient. MS less likely as he is a male, a little older than usual age range for MS, and does not describe h/o episodic neurological symptoms. encephalitis would more likely present with some other symptoms like fever, AMS, confusion, etc. wernicke’s would likely also have other symptoms like memory loss or confusion, and would have elicitied h/o chronic ETOH use with poor dietary intake/nutrition.