3/12 Presby report: vertigo, MS/NMO

Thanks to Rebecca Wang for presenting a cool case of a middle aged man who presented with dizziness/vertigo and was found to have demyelinating brain lesions, felt to be MS vs NMO.

Differentiating between dizziness and true ‘room spinning’ or vertigo is important. You can categorize the complaint of dizziness into pre-syncope, disequilibrium, lightheadedness and true vertigo (which should be accompanied by the ‘room spinning’ sensation).

Questions to ask when investigating vertigo

  • How long the vertigo has been going on (longer = more concerning for central)
  • Things that provoke the symptoms (changes in head position, head trauma, loud noises)
  • Tinnitus/hearing loss
  • Recent URIs
  • Focal neuro deficits
  • Once vertigo is established, try to differentiate peripheral and central causes.  Central causes are often the more acutely concerning because they include mass lesion, stroke, as well as demyelinating conditions, etc
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Here’s an excellent table that helps distinguish central vs peripheral vertigo. Note that no one test or feature reliably distinguishes the two. Initial Evaluation of Vertigo, AAFP.

In one study, the presence of vertigo upon waking up in the morning was predictive of a peripheral cause.

Provoking factors may also be helpful in identifying a diagnosis.

  • Symptoms provoked by…
    • positional changes: BPPV
    • recent URI: vestibular neuronitis or labrynthitis
    • migraine triggers: vestibular migraine
    • straining, recent head trauma, loud noises: perilymphatic fistula
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The Dix-Hallpike maneuver for positional vertigo is performed by the examiner, who stands at the head of the bed. As the patient is supported and lowered into a position whereby his or her rotated and extended head hangs off the end of the examining table, the examiner observes for nystagmus. In this view, the patient’s head has been rotated to the left and expresses nystagmus with a slow response to the right and a rapid response the left. Repeating the maneuver with the head rotated in the opposite direction would reverse the direction of the nystagmus. A maneuver (with positive indication) will reproduce the patient’s symptoms.

Remember that the Romberg test is generally not a test for cerebellar function but for peripheral neuropathy.

Finally, the HINTS (Head Impuse, Nystagmus, Test of Skew) exam! This test is useful for distinguishing brainstem and cerebellar ischemia from vestibular neuritis or other peripheral causes of vertigo and is most helpful in patients who have had continuous feelings of vertigo or dizziness. It is not useful in patients with momentary position-related transient vertigo (often benign positional vertigo) or those with TIAs who are not dizzy when examined

HINTS was found to have a sensitivity of 96.5% and specificity of 84.4% in identifying central causes of vertigo, which was much better than ABCD2. This is even better than MRI

To summarize: a REASSURING HINTS exam is ALL of the following 1) unidirectional nystagmus 2) no vertical skew 3) abnormal head impulse test (abnormal = nerve problem, not brain problem).

Neuromyelitis optica (NMO)

NMO is a demyelinating disease that primarily targets the optic nerves and spinal cord. 55-85% of brain imaging is normal, although patients can have nonspecific optic nerve enhancement and C/T spine enhancement. Almost all lesions are spinal cord or in cranial nerves.

Hallmark features

  • bilateral or rapidly sequential optic neuritis
  • transverse myelitis (leading to limb weakness, sensory loss, bladder dysfunction)
  • Trunk/leg pain
NMO vs. MS
  • NMO was originally classified as part of MS
  • NMO is now recognized as a separate disease because they do worse than MS patients.  NMO is also associated with IgG antibodies to Aquaporin 4, although this is neither necessary nor sufficient for diagnosis
  • NMO shares many features with MS, such as the development of lesions over time and space
  • More acute than MS; progressive decline over several years w/ a higher mortality than MS
  • NMO lesions are almost exclusively brain stem and cranial nerves, and may include transverse myelitis

Differentiating NMO from MS is based on differences with respect to clinical course, pathophys, and response to MS drugs. Some radiographic features that are more suggestive of MS than NMO:

• Lesions adjacent to lateral ventricle
• Inferior temporal lobe white matter lesions
• Ovoid (ie, “Dawson finger”) periventricular lesions
• U-fiber juxtacortical lesions


Labuguen R. Initial Evaluation of Vertigo. AAFP 2006.



1/2 PPMC Report: CMV Retinitis and Ocular Neurosyphilis

Thank you to Dr. Gluckman for your expert insight as we discussed a really interesting case of a 52 year old male who was diagnosed with ocular neurosyphilis. Our patient has a history of AIDS diagnosed when he presented with CMV retinitis 7 years ago, after which he was adherent with ARTs until several months ago – then six weeks prior to presentation he restarted ARTs. He reported approximately one week of increased floaters and decreased visual acuity in his left eye. He was evaluated in ophthalmology clinic and found to have decreased left visual acuity as well as optic nerve edema and inflammation, and was admitted for further workup.

A few words about CMV retinitis — we do not see it very much now that there is effective combination ART, but before that was available this was pretty common in AIDS patients. It is usually seen with very low CD4 counts (<50), and can be unilateral initially. It is thought to represent hematogenous spread of CMV, and then causes full-thickness retinal edema and necrosis, which spreads centrifugally and causes visual symptoms that depend on the involved areas. When the inflammatory phase is resolved, it leaves behind thin atrophic retinal scars, which are prone to retinal detachment and tears.

The diagnosis is made based on characteristic findings on dilated fundoscopic exam. Notable, CMV viremia is detected in less than half of the patients with CMV retinitis, but negative CMV IgG makes this diagnosis much less likely.

Treatment is aimed at stopping spread to unaffected areas of the retina. If there is immediate threat to vision, intravitreous injection of foscarnet or gancyclovir is indicated. If not, oral valgancyclovir can be used, and many providers will treat for two weeks before starting ART to avoid a recovery uveitis phenomenon.

Our patient did not have characteristic CMV retinitis findings on his fundoscopic exam. He was noted to have a maculopapular rash and a history of syphilis, so clinical concern for neurosyphilis was high. His RPR ultimately came back with a very high titer — remember that if clinical suspicion for syphilis is high and RPR comes back negative, you may need to ask the lab to run a prozone assay. This means additional dilutions to ensure that antigen is not so high as to preclude formation of crosslinking antibodies (resulting in a false negative). Of note, CSF VDRL does NOT need to be positive to have a diagnosis of neurosyphilis. This patient was diagnosed with neurosyphilis and started on Penicillin G for treatment, along with steroid eye drops to help with swelling.

10/31 HUP Report: ALS

Thanks so much to our experts from the neurology department for coming to discuss a case of a 61 year old female without significant past medical history who presented with lower extremity weakness, progressive over several months, without associated sensory deficits. We broke our differential down by first thinking through the anatomy of the neuromuscular circuitry, which involves the upper motor neuron synapsing on the lower motor neuron, which interacts with the muscle cells through the neuromuscular junction. Here is a limited differential that we discussed:


This patient had both upper and lower motor neuron findings, and was diagnosed with ALS. Depending on location of affected nerves, presenting signs/symptoms of ALS may be weakness, fasciculations, atrophy similar to this patient. Patients can also present with hypercapnea due to diaphragm involvement, or bulbar signs such as slurring, dysphagia.


Currently, most of the treatment for ALS patients involves symptomatic management, as understanding of ALS pathophysiology and development of targeted interventions is still a topic of research. Riluzole is one medication that has been shown to have benefit in ALS patients, with quoted benefits of 2-3 month increase in survival and delaying respiratory failure. Interventions such as percutaneous endoscopic gastrostomy tubes and ventilator support are very personal decisions for patients and their families.

ALS patients benefit from a multidisciplinary care team, including physician specialists as well as respiratory therapists, occupational and physical therapists, social support team and others to provide best comprehensive care for themselves and their families.


8/24 PPMC Report: Vertigo

Today’s case was a 47 yo F with congenital lymphangiectasia presenting with vertigo.

Obviously — the first thing we discussed was a quick review of congenital lymphangiectasia. It’s an exceedingly rare disease in which there are dilatation of intestinal lacteals resulting in lymph leakage into the small bowel. This disease is responsible for a protein-losing enteropathy leading to lymphopenia (CD4 count 36), hypoalbuminemia, and hypogammaglobulinemia.

Next we did a review of central vs. peripheral vertigo and how to differentiate between the two based on history and exam. While it’s not COMPLETELY black and white, the table below gives general guidelines to differentiate between the two.vertigo


Based on our patient’s presentation with vertical and horizontal nystagmus and positive skew deviation she was referred for MRI with c/f a central lesion.

Her MRI demonstrated multiple rim-enhancing brain lesions and we discussed the broad differential for an immunocompromised patient — including bacteria (abscess v. TB v. syphilis/gummas), fungal (crypto v. aspergillosis, v. histo v. coccidio v. actino v. mucor), Parasitic (toxo), Inflammatory (sarcoid v. whipple’s v. MS v. lupus v. PML v. Behcet’s) and Neoplastic (metastases vs. primary CNS lymphoma vs. GBM vs astrocytoma).

She had a biopsy that demonstrated primary CNS lymphoma.

8/3 (HUP): stroke pearls, IA thrombectomy

Many thanks to Dr. Steve Messe for walking us through some amazing advances in stroke care- namely intra-arterial thrombectomy.


  1. The NIH stroke scale (<5 mild, 5-15 moderate, >15 severe) is used to assess stroke severity, and has great inter-rater reliability. It’s less sensitive for brainstem and cerebellar infarcts and those in the primary hand motor cortex (since it only assesses proximal arm strength). It’s a great predictor of short and long-term outcome.
  2. Mechanical intra-arterial (IA) thrombectomy has been shown in multiple trials (MR CLEAN, ESCAPE, REVASCAT) to be safe and effective, with NNTs ranging from 3-7 (!) to prevent bad neurologic outcomes
  3. According to guidelines, IA thrombectomy technically should be used within 6h of proximal large artery occlusion in patients with an NIHSS >6. That being said…
  4. …unpublished data from the DAWN study suggests that IA thrombectomy up to 24 hours after occlusion had significantly better neurological outcomes- a 73% relative risk reduction in disability in those that received thrombectomy!
  5. A brief note on tPA: the old teaching used to be that older patients (>80) didn’t benefit from tPA, but recent analyses suggest that older patients may actually benefit even more than younger patients. Also remember that tPA can now be used up to 4.5 hours after symptom onset (or last known normal)
  6. Remember that as with anything, tPA use is a risk-benefit decision; even if someone has a low NIHSS, they may still benefit from tPA if they would otherwise be disabled (ex: distal arm/hand immobility in a laborer, artist, etc)

Lastly- the paper of the day. It suggests that combining Vitamin C and thiamine with hydrocortisone in septic shock may come with a mortality benefit, but this is clearly not a randomized trial and thus more experience is probably needed. See this excellent blog post on PulmCrit for a more detailed analysis.

  1. Marik, P. et al. 2017. Hydrocortisone, Vitamin C, and Thiamine for the Treatment of Severe Sepsis and Septic Shock A Retrospective Before-After Study. Chest. PMID 27940189

8/3 PPMC Report: Bacterial Meningitis

Today we covered the often thought of (but less frequently diagnosed) bacterial meningitis and a case diagnosed and treated by our own Dr. Ferrante!

We discussed pattern recognition (“thinking fast”) and the benefits in this case. We reviewed the data on the signs and symptoms at presentation for meningitis including that having fever, neck stiffness and AMS is only 46% specific; however >95% of patients who were eventually diagnosed with bacterial meningitis had 2+ of these clinical findings (1).

We chatted about who needs a HCT prior to LP (2):

  1. Immunocompromized patients
  2. History of CNS disease (masses, stroke, focal infection)
  3. New onset seizure (within the last week)
  4. Papilledema
  5. Abnormal level of consciousness (inability to answer 2 consecutive questions or follow 2 commands)
  6. Focal neuro defect

We also reviewed the data for dexamethasone (10mg Q6H x 4 days) started prior to antibiotics in adults. The 2002 NEJM article (3) demonstrated decreased unfavorable outcomes in the dexamethasone group as compared to the placebo (15% vs. 25% with RR 0.59). This was especially pronounced in those with S. pneumo meningitis (26% unfavorable outcome vs. 52% in placebo group). While the data for steroids improving outcomes with Neissieria is lacking, until you have the bug back, it’s worth the steroids!

Finally, we reviewed the CDC guidelines for pneumococcal vaccines and the data that demonstrated that PCV vaccination DECREASES S. Pneumo meningitis (54% reduction in incidence in those >65 during) (4).

  1. Attia J et al. Does This Adult Patient have Acute Meningitis? JAMA 1999;281(2): 175-181.
  2. Tunkel AR et al. Practice Guidelines for the management of bacterial meningitis. CID 2004;39:1267-84.
  3. De Gans J et al. Dexamethasone in Adults with Bacterial Meningitis. NEJM 2002;347:1549-1556.
  4. Hsu, HE et al. Effect of pneumococcal conjugate vaccine on pneumococcal meningitis. NEJM 2009;360:244-56.