Category Archives: Neurology

VA Ambulatory Report 11.28.17 – When your patient is seeing double – Diplopia and Stroke Pearls

Thanks Amanda for sharing your case of a middle aged male with a past history of diabetes and HTN presenting with diplopia found to have a sixth nerve palsy due to a pontine infarct.

Diplopia Pearls   Screen Shot 2017-10-11 at 12.58.11 PM

  • Your approach to diplopia should be the same as we approach any neurologic deficit –  Localize the lesion along the anatomic pathway
    • Central
    • Peripheral cranial nerve
      • Both a mononeuropathy or external compression of the cranial nerve
    • Extraocular muscles
    • neuromuscular junction
    • Globe (retina, cornea, lens)
  • Monocular diplopia vs. binocular diplopia
    • Binocular diplopia: If the diplopia is present only when both eyes are open then the diplopia is due to ocular misalignment
    • Monocular: Most often due to diseasse of the cornea or lens, less commonly the retina or cerebral lesion
  • Extraoccular muscle weakness can be subtle. LT pearl: when testing EOM, move your finger fast as this will be more likely to demonstrate subtle weakness than slow movements.
  • Cranial nerve palsies
    • 3rd nerve: longest cranial nerve after exiting the brainstem, more commonly affected by compression from an aneurysm or tumor.  When a third nerve palsy is due to microinfarction from diabetes it typically spares the pupil.  Therefore pupillary defects should raise your suspicion for intracranial lesion
    • 4th nerve: Most prone to injury from trauma and more rarely affected by microinfarction
    • 6th nerve: Most commonly affected

Check out this excellent chart from EB Medicine reviewing CN III, IV, and VI palsies.

Screen Shot 2017-11-30 at 1.49.47 PM

Stroke Pearls    Screen Shot 2017-10-11 at 12.58.11 PM

  • Code stroke: time sensitive interventions may be changing in the future
    • The latest trials are showing benefit to endovascular intervention in patients with large vessel occlusion within 6 hours of symptoms onset even after tPA administration.
    • Newer trials are investigating endovascular intervention up to 24 hours after symptom onset
  • Risk of future stroke after TIA: Use the ABCD2 score
    • This helps estimate the risk of stroke within the next 2 days after TIA
    • In patients with a risk < 1%, outpatient work-up may be appropriate
      • Goal is to get work=up completed within 24 hours

Evernote link:


VA ICU Report 5.26.17

Morning, some good neuro critical care action at the VA this week, thought we’d cover something today not yet covered on the blog: non-invasive estimations of elevated ICP, namely fundoscopy and ocular ultrasound

Dan Reiss correctly pointed out the acute finding in elevated ICP on an ocular ultrasound is the optic nerve sheath diameter, a handful of smaller studies (n in each about 40-60) have evaluated the correlation between nerve sheath diameter and MRI/CT findings of elevated CT, Sensitivity and Specificy depends on the cutoff chosen, in adults typically a ONSD of >4.5mm is considered too high, which yields a sens and spec of around 70-80% for the detection of elevated ICP. The challenge here, as with fundoscopy, is inter-observer variability presumably due to operator characteristics. Here is a review paper on non-invasive determination of ICP.

Other quick pearls

#generally opt for beta blockers and ccb in the acute treatment of hypertensive emergency due to the theoretical concern for cerebral vasodilation with nitro/hydral, nicardipine and esmolol are good options

#PRES, covered elswhere, stands for posterior reversible encephalopathy syndrome, though not always posterior or reversible. Remember, MRI is the diagnostic test for PRES.


ZSFG can’t be MIF’d by penile lesion ddx & indications for sgy in endocarditis

At the General, we give you a little bit of this and a little bit of that in report. And same thing goes for the chiefs’ blog. This is a quick run-through of a few recent legendary reports!


In Neuro Report today, we crushed, I mean discussed, hypercarbic respiratory failure and the role of neuromuscular causes for it. We were joined by neurology guru, Andy Romeo, and here are a few of his pearls:

-Whenever you come across someone reporting dysphagia, make sure to ask about other bulbar sx’s
-In a patient with increased work of breathing in whom you’re considering if diaphragmatic weakness is playing a role, check neck flexor strength to assess if a new neuromuscular weakness is present
MIF & VC are the confrontational tests for the diaphragm. To remind ourselves about those two entities:

  • For Vital capacity (VC) and Mean inspiratory force (MIF), there is the 20-30 rule
  • VC: deep breath and exhalation maximally into spirometer; goal is at least 20cc/kg
  • MIF: inhalation against a closed valve with negative force recorded; goal is “more negative” than -30 cmH20. -60cmH20 is expected or what is associated with weak cough in NL person


In a recent ID report, we discussed the well-known penile lesion ddx and added in a lesser known branch point of the *PRURITIC* penile lesion. The following is a non-exhaustive (and likely with much overlap) summary of what we came up with:

PAINFUL penile lesion

  • Chancroid/H. ducreyi
  • SJS/TEN drug lesion
  • SCC
  • Traumatic lesion/entrapment injury
  • Ulcers in s/o foley
  • HSV
  • Paraphimosis

 PAINLESS penile lesion

  • Syphilis
  • LGV (Of note, the lymphadenopathy *IS* painful in Lymphogranuloma venereum; LGV caused by L1, L2, L3 serovars of Chlamydia trachomatis)
  • Granuloma Inguinale (uncommon infection caused by K. granulomatis)
  • HIV
  • HPV
  • Pearly penile papule

*PRURITIC* penile lesion

  • Fixed drug reaction, DRESS/DHR
  • Yeast
  • Infestation-scabies/pubic lice
  • HSV
  • Behcet’s

 So how do we diagnosis LGV? Does our usual urine test work??????
Lisa Winston teaches us:

Turns out the usual Chalmydia culture or the more commonly ordered/sensitive NAAT test will be positive in LGV as the serovars will be picked up—it just won’t specify that it detected the L1-3 serovars. Usually when the sx’s are classic, empiric tx (longer course) is initiated. If you want a definitive dx, you can talk to colleagues at communicable dz and public health to see if need to send serology or special PCR to the SF public health lab (and then potentially to state’s public health lab or CDC).


Lastly, Mike and Carine presented a patient in intern report with MV endocarditis 2/2 MSSA where we discussed the role of early surgical intervention in infective endocarditis.

There is a fantastic 2013 NEJM Infective Endocarditis review article by Hoen and Duval that breaks down the indications for surgery into three big categories: heart failure, uncontrolled infection, and prevention of embolic events. Or in image form:

indications for sgy

For those of you who want more…

Punag, one of the cardiology fellows, passes on the following for the ACC/AHA class indications for surgical intervention:

Early surgery is recommended for patients with complicated infective endocarditis (IE), but data from randomized trials are scarce. The following are points to remember about the timing of surgery among patients with IE:

  1. The main indications for early surgery in IE are heart failure, uncontrolled infection, and prevention of embolization. The reduction in mortality with surgery is greatest among patients with IE and moderate to severe heart failure.
  2. Heart failure. The European Society of Cardiology (ESC) guideline (2009) recommends emergent surgery for heart failure with refractory pulmonary edema or cardiogenic shock (Class I), or urgent surgery for persistent heart failure with signs of poor hemodynamic tolerance (Class IIa). The American Heart Association (AHA)/American College of Cardiology (ACC) guideline (2014) recommends early surgery for valve dysfunction causing heart failure (Class I).
  3. Uncontrolled infection. The ESC guideline recommends urgent surgery (Class I) for evidence of uncontrolled infection defined as either abscess, fistula, or pseudoaneurysm; or for an enlarging vegetation, persistent fever, or positive blood cultures after 7-10 days of appropriate therapy. The AHA/ACC guideline recommends early surgery (Class I) for evidence of persistent infection, heart block or abscess, or a resistant organism ( aureus, fungi).
  4. Prevention of embolization. The ESC guideline recommends urgent surgery for a vegetation >10 mm with previous embolization or other surgical indication (Class I), or for isolated vegetation >15 mm and feasible valve repair (Class IIb). The AHA/ACC guideline recommends early surgery for recurrent emboli and persistent vegetations despite appropriate antibiotic therapy (Class IIa); or a large mobile vegetation on a native valve (Class IIb).
  5. Neurological complications. Patients with a neurological complication may have other indications for early surgery. However, early surgery may pose a significant risk for perioperative neurological deterioration (related to anticoagulation potentiating the risk of intracerebral bleeding, and to hypotension during cardiopulmonary bypass aggravating neurological ischemia and edema).
  6. Prosthetic valve IE. Prosthetic valve endocarditis is the most serious form of IE, and more difficult to treat using antibiotics alone. In general, current guidelines support consideration of a surgical strategy for high-risk subgroups with prosthetic valve IE, including patients with heart failure, abscess, or persistent fever.
  7. Definitions of early surgery. There is no consensus as to the optimal timing of early surgery. The ESC guideline classifies surgical indications in IE as emergent (within 24 hours), urgent (within a few days), and elective (after 1-2 weeks of antibiotic therapy). The AHA/ACC guideline defines early surgery as occurring during the initial hospitalization and before completion of a full therapeutic course of antibiotics.


Evernote link:


Hoen B, Duval X. Infective Endocarditis. N Engl J Med 2013; 368:1425-1433April 11, 2013DOI: 10.1056/NEJMcp1206782

Ramsay-Hunt Syndrome – updated

Thanks to Adam Tabbaa and Megan Lockwood for presenting a 40 y/o healthy man who presented with multiple cranial neuropathies and ear vesicles concerning for Ramsey Hunt Syndrome
What is Ramsey Hunt syndrome?
  • classically, Facial nerve paralysis + Zoster oticus (zoster lesions in the ear) +/- hearing loss
  • Pathophysiologically – Zoster reactivation in the geniculate ganglion.
This case hinged upon identifying the patient’s cranial neuropathies and trying to identify an atomic location whether those cranial nerves ran together. So here’s a quick refresher on the facial nerve and the geniculate ganglion.
facial nerve 2
  • The facial nerve exits the brain stem, courses through the temporal bone, then forms a knee-shaped ganglion at its exit

facial nerve 1.png

  • As this less detailed image shows. The facial nerve runs *extremely close* to the ear when exits the temporal bone. So when VZV reactivates in the geniculate ganglion, it travels transaxonally through the nerves to skin around the ear, causing vesicles there.
    • Most VZV reactivation in the face is in the trigeminal nerve, which is why we are so accustomed to seeing zoster in a V1, V2, or V3 distribution. Turns out it can affect whatever nerve it feels like.
  • When the facial nerve runs next to the ear, it also runs in close quarters with the vestibulocochlear nerve. So Ramsey-Hunt can also include deafness or vestibular symptoms and for this reason is considered an ENT emergency.
  • Ramsey Hunt is usually managed with acyclovir + steroids. A cochrane review showed no evidence of benefit for acyclovir but the overall evidence quality was poor.
  • Ramsey Hunt, a peripheral nerve manifestation of zoster, should be distinguished from CNS zoster which can be devastating. Zoster encephalitis, if it affects the brain stem, can also pick off specific cranial nerves. Because our patient also had deficits in non-adjacent cranial nerves (IX and X), we suspected brainstem disease.
Multiple Cranial Neuropathies
  • So interesting! So serious. More pearls about them here:
Kleinschmidt-DeMasters BK1, Gilden DH. Varicella-Zoster virus infections of the nervous system: clinical and pathologic correlates. Arch Pathol Lab Med. 2001 Jun;125(6):770-80.
Whitley RJ1.A 70-year-old woman with shingles: review of herpes zoster. JAMA. 2009 Jul 1;302(1):73-80. doi: 10.1001/jama.2009.822. Epub 2009 Jun 2.
Rebecca Miller-Kuhlmann, MD. My very favorite neurologist who texted me many pictures of cranial nerves.

ZSFG Pearls: Abx associated encephalopathy and PAN

From neuro report and intern report at ZSFG, we have some juicy pearls coming your way…

Top Pearlzzzz:

  • From Arturo, neurologist extraordinaire: If the patient is older (>70), it is a normal variant for them to have lost/diminished ankle reflexes. It’s not just our (amazing) exam technique
  • Visual hallucinations are generally not from a primary psychiatric etiology. Consider delirium causes with a special emphasis on primary neurologic ones
  • See a patient picking at their bed sheet? Consider this to be “Lint picking behavior” or CARPHOLOGIA which tends to be strongly associated with delirium


We also discussed antibiotic-associated encephalopathy (AAE) in the form of a case of an older man receiving Cefepime for sepsis of likely urinary etiology who developed confusion and carphologia on HD3. The delirium work-up did not show an obvious cause, and the MRI was nL.  His sx’s were attributed to AAE and improved once Cefepime was stopped. A recent review in Neurology (see below) discussed three clinical phenotypes of AAE to know:

  • 1) Encephalopathy commonly accompanied by seizures or myoclonus arising within days after antibiotic administration
    • Causative agents: Cephalosporins and penicillin–>mechanistically it’s thought that the B-lactam ring interferes with GABA
  • 2) Encephalopathy characterized by psychosis arising within days of antibiotic administration
    • Causative agents: Fluroquinolones, macrolides, and procaine penicillin
  • 3) Encephalopathy accompanied by cerebellar signs and MRI abnormalities emerging weeks after initiation of antibiotics
    • Causative agent: Metronidazole



From Intern report today and the fascinating discussion on Vasculitis. Let’s make sure we pass on that amazing diagram of vessel size and corresponding entities:

vasculitis outline.jpg

A bit more about Polyarteritis Nodosa, specifically

  • Def’n: ANCA- negative, necrotizing vasculitis involving medium- sized arteries
  • Pathogenesis: immune complex deposition within the intima of medium- sized arterial vasculature; ~1/3 cases are associated with HBV but most are idiopathic
  • EPI: Rare, affecting fewer than 30 people per million
  • Clinical Presentation:
    • Systemic symptoms (fatigue, arthralgias, weight loss, or fever)
    • Multisystem involvement
      • Neurologic symptoms (peripheral neuropathy, mononeuritis multiplex)
      • Skin involvement (nodules, purpura, ulcerations or livedo reticularis, digital ischemia)
      • Renal involvement (but not associated with glomerulonephritis!)
      • GI: mesenteric ischemia (post-prandial abdominal pain)
      • Notably spares the pulmonary vascular bed
  • DX: Biopsy of nerve, skin, or muscle will show necrotizing arterial inflammation. ***Avoid the kidneys and liver for biopsy given the risk of hemorrhage or vascular rupture***


  • Visceral angiography will show microaneurysms in the renal, mesenteric or hepatic vasculature
  • Labs are supportive but not diagnostic
    • Elevated inflammatory markers
    • Usually ANCA negative (presence MPO or PR3 favors another dx)
    • Normal complements
    • low C4 suggests cryoglobulinemic vasculitis
    • low C3 and C4 suggests SLE
      • Treatment:
        • Glucocorticoids alone +/- cyclophosphamide or azathioprine
        • ACE inhibitors
        • Poor prognosis for patients with untreated PAN
        • 5-year survival rate of 13%; death is often a consequence of renal failure, MI, or stroke
        • With appropriate therapy, 5-year survival rate ~ 80%


      Reference: Bhattacharyya et al. Antibiotic-associated encephalopathy. Neurology. 2016 Mar 8;86(10):963-71. doi: 10.1212/WNL.0000000000002455. Epub 2016 Feb 17. PMID: 26888997


Neuro report! – approach to myopathy

Thank you to Christy for presenting an outpatient case of a patient with progressive proximal>distal lower extremity weakness concerning for myopathy and Kevin Keenan for acting as our neurology consultant.
UpToDate had the best review article I could find on a general approach to myopathies. Here’s a synthesis of that + Kevin’s teaching today.
Weakness framework 2.0
Step 1: distinguish asthenia from true weakness
Step 2: Weakness is best characterized by localization and pattern
  • brain
  • “electrical” (migraine or seizure)
  • spinal cord
  • CSF (like a meningitis)
  • anterior horn cell
  • peripheral nerve
  • neuromuscular junction
  • myopathy
  • progressive
  • monophasic
  • static
  • relapsing/remiting
  • improving
Myopathy ddx
  • When i hear the word “myopathy” my first instinct is to think about inflammatory causes. Other etiologies are much more common
  • statins, alcohol, stimulants
  • cushings
  • hypothyroid
  • vitamin D deficiency
  • primary myositis (dermatomyositis, polymyositis, inclusion body myositis)
  • scleroderma
  • SLE
  • overlap syndromes
  • glycogen storage disorders etc
  • suspect these when patients report pigmenturia with exertion or other stress
muscular dystrophies
  • paraneoplastic
First pass workup
  • An awesome neuro exam
    • pay careful attention to respiratory muscle weakness and cranial neuropathies, as these may signal a more emergent condition
  • serum: CBC, chem 10, CK (can also consider LDH and AST if your suspicion is high), ANA, TSH, Vitamin D, hepatitis serologies
    • if very suspicious for an inflammatory myopathy, consider SSA/SSB, anti-SM, anti-RNP, anti Jo-1.
  • Urine: UA to look for myoglobinuria
  • What about an EMG/Nerve conduction study?
    • In patients with true myopathy without an obvious reversible cause (like statins) EMG/NCS is necessary to confirm the diagnosis and should be ordered early in the diagnostic workup.
Bonus pearls
  • Grip strength: Kevin told us to abandon grip strength in our screening motor exam. It is selective preserved in UMN weakness and very hard to grade objectively. When doing a screening motor exam, try pronator drift + one distal muscle group (like wrist extension) + one proximal muscle group (like deltoids)

Tuberculous Meningits and CNS Whippel’s Disease

Today, we discussed a case of an elderly woman presenting with recurrent, cryptogenic stroke. We had an interesting discussion on approach to recurrent strokes, and discussed the diagnosis of tuberculous meningitis.

Top Pearls from Today:
A. Serial LP’s are often necessary to confirm diagnosis of various CNS infections, including tuberculous meningitis. CNS AFB yield increases from 37% (with 1 LP) to 89% (with 4 LP’s)!

B. If clinical suspicion is high for tuberculous meningitis and AFB staining is negative, send nucleic acid testing of CSF!______________________________________________________

Interested in more details?

Tuberculous Meningitis

  • 1% of all cases of TB and 5% of all extrapulmonary disease in immunocompetent individuals
  • Three discernible phases of TB meningitis 1) prodromal phase (2-3 weeks): malaise, headache, low-grade fever 2) meningitic phase: meningismus, headache, vomiting, lethargy, confusion, cranial nerve involvement 3) paralytic phase: stupor, coma, seizures, hemiparesis. If untreated, death ensues.
  • Diagnosis – can be quite difficult! – CSF: Characteristic CSF findings of low glucose, elevated protein, mononuclear pleocytosis (however, early in the course of illness, cellular reaction is often atypical. Needs serial CSF examinations, which may show a change to a lymphocytic cellular pattern!) – AFB smear and culture: in general, a minimum of 3 serial LPs should be performed, as diagnostic yield can increase from 37% on LP#1 to 89% on LP #4! – Nucleic acid tests: Xpert MTB/RIF assay should be submitted in the setting of high clinical suspicion and negative AFB staining.

CNS Disease in Whipple’s

  • Whipple’s Disease is a rare entity that may be a mimicker of many different illnesses!
  • When to suspect Whipple’s? – Think about 4 cardinal manifestations: arthralgias, diarrhea, abdominal pain, weight loss – other clinical syndromes that raise suspicion include: FUO, chronic serositis, progressive CNS disease or early onset cognitive deficits, myoclonus or ophthalmoplegia, generalized lymphadenopathy!
  • Neurologic involvement has been reported in 10-40% of cases with Whippel’s disease (isolated CNS infection with T. whipplei can rarely occur).
  • Most commonly, CNS involvement  is asymptomatic – diagnosed usually by PCR detection of T. whipplei in the CSF!
  • Various clinical syndromes may occur with primary Whipple’s disease of the brain 1) Multifarious neurologic symptoms and signs: seizures, ataxia, eye movement disorders, amnesic syndrome, SIADH, meningoencephalitis, dementia 2) Focal neurologic symptoms secondary to a solitary mass lesion

Evernote Link: