Dr. Priya Sharma, one of our phenomenal senior residents, presented a “hot case” this morning: a middle-aged man with rapidly progressive dementia characterized by language deficits, behavioral changes, paranoia/obsessions, and tremors that evolved over the course of several months. After a initial workup ruled out reversible medical etiologies, the patient was ultimately transferred to the Neurology service for further workup.
We were incredibly fortunate to have been joined by Dr. David Coughlin, our movement disorder specialist extraordinaire, who reviewed key historical features and exam findings while evaluating a rapidly progressive dementia syndrome. It is important to obtain collateral information, assess the patient’s psychosocial milieu, and rule out any contribution from delirium. The differential diagnosis for rapidly progressive dementia is quite broad and includes vascular, infectious, toxic-metabolic, autoimmune, malignant, iatrogenic, neurodegenerative, and systemic/structural/seizure-related causes. If initial laboratory and imaging studies fail to disclose an etiology, additional serum/CSF, advanced imaging, and electrographic studies should be considered.
Several studies are still outstanding for our patient. The working differential includes Creutzfeldt-Jakob disease, autoimmune encephalitis (e.g., limbic encephalitis, anti-LGI1 encephalitis), and an atypical presentation of frontotemporal dementia. We anticipate some of these studies will return in the next few weeks. Stay tuned for an update!
Thank you to Dr. David Coughlin and the Neurology team for participating in this multidisciplinary morning report and for taking wonderful care of this patient.
Medical Spanish Word of the Day: el cerebro = brain
During our Hillcrest morning conference today, we learned about the unfortunate case of an otherwise healthy young man who developed herpes zoster ophthalmicus. He presented with severe right-sided eye pain that began a few days after the appearance of confluent vesicles in the CN V1 and V2 distributions (i.e., ophthalmic and maxillary divisions of the trigeminal nerve). He also had an intense, burning pain in the area of the rash. We reviewed important differential considerations, including orbital cellulitis, keratitis, conjunctivitis, anterior uveitis, optic neuritis, dry eye syndrome, acute angle-closure glaucoma, and cluster headache. Ultimately, an ophthalmologic exam revealed pseudodendrites consistent with VZV keratitis. A search for provoking factors for VZV reactivation, including an HIV test, was unrevealing. His symptoms improved after treatment with IV acyclovir, hydration, IV analgesics, and antimicrobial eyedrops.
Our ID fellow extraordinaire, Dr. Rehan Syed, guided us through a whirlwind tour of the potential ocular and neurologic complications of herpes zoster, including herpes zoster ophthalmicus and acute retinal necrosis, and their initial management. Because some of these conditions can be sight- or life-threatening, early recognition is critical. Special thanks to Dr. Syed for sharing his insights on these important conditions!
Key Learning Points:
Uncomplicated herpes zoster infection is treated with a 7-day course of an oral antiviral such valacyclovir.
If there is any concern for CN V1 involvement or visual disturbances, call ophthalmology for an urgent evaluation!
If there is ocular or CNS involvement, a more prolonged treatment course with IV acyclovir is recommended.
Medical Spanish Word of the Day: la culebrilla (lah koo-leh-BREE-yah) = shingles
Thursday morning, Brian Coburn, one of amazing second year Med/Peds residents, presented an interesting case of a 35-year-old patient who presented with hypoactive delirium. The patient’s medical history was significant for epilepsy and opioid use disorder. We started our investigation of possible causes of the patient’s encephalopathy by going through the AEIOUTIPS mnemonic of common causes of altered mental status that can be investigated with basic history, exam, labs, and imaging. Our expert discussants from the Toxicology services provided us a comprehensive overview of the approach to hypoactive toxidromes and judicious interpretation of urine tox screening. After the initial work-up was unrevealing, the team pursued additional investigation with lumbar puncture, EEG, and MRI without contrast that were also negative. The team asked our friends from psychiatry to help elucidate whether the patient’s symptoms may be due to an underlying psychiatric disorder, but the determination was made that a primary medical disease was still the most likely cause.
The patient’s condition continued to progress, and an MRI with contrast ultimately revealed acute, diffuse white matter lesions. Per our radiology colleagues, the differential for these findings included infection/post-infectious causes (viral encephalitis, acute disseminated encephalomyelitis, acute hemorrhagic leukoencephalitis), autoimmune encephalitis, and toxic leukoencephalopathy. Following a repeat LP that was negative for a broad array of infectious, autoimmune, and malignant/paraneoplastic studies, we were left with a diagnosis of opiate-induced toxic leukoencephalopathy. The disease presentation is extremely variable, ranging from minor cognitive impairment, easily confused with psychiatric illnesses, to severe neurological dysfunction. Our toxicologist reminded us that some of these findings can also be seen with potential transient, out-of-hospital anoxic episodes. While there is no proven treatment for toxic leukoencephalopathy, antioxidant therapy with Coenzyme Q10, Vitamin E, and Vitamin C has been used to treat some patients with variable results in the past. The patient received this treatment and spontaneously improved after several days of therapy.
Thank you to Brian, our expert discussants, and our residents for working through the differential of hypoactive delirium and the sequential investigations we needed to get to the bottom of this fascinating case.
Today we discussed a young man who was establishing care and had a concerning recent hospitalization for R sided facial droop and slurred speech. His symptoms thankfully self resolved but he had been diagnosed with a possible L corona radiata stroke on MRI. We went over our differential for facial palsy then based on our review of his imaging and his work-up of symptoms, we had some concerns that he had MS. We reviewed as group our illness script for MS and point out why he may not fit our usual model patient for this disease process. Then we discussed the diagnostic criteria (source: https://pn.bmj.com/content/19/1/64) and further tests we could order for the work-up of this patient.
One diagnostic schema for thinking of causes of facial palsy is dividing it into central/supranuclear vs peripheral/nuclear causes. You can differentiate the two by looking at the patient’s forehead to see if innervation is preserved or affected (with peripheral causes leading to loss of innervation)
MS is often a relapsing and remitting autoimmune process that can affect the central nervous system at many levels: with optic neuritis, myelitis, INO being some common symptoms
MS can be diagnosed using the MacDonald Criteria which takes into account clinical symptoms and imaging over time and in regards to specific neurologic distribution. Know that only specific patterns of MRI white matter defects are suggestive of MRI and that evoked potential testing can be another way of looking for demyelination.
Today our amazing R3 Allen Jiang led us through a case of a elderly man with a history of HTN, HFpeEF, Afib and DM who came in with SOB and was found to have severe hypertension.
He had a headache and then developed respiratory failure and had a seizure and thus we were concerned for hypertensive emergency specifically for an intracranial process such as PRES.
We went over the classifications for severe hypertensive and also the mechanism of autoregulation for blood pressure for the brain to remind of how end-organ damage may manifest itself there.
Our expert discussant, Dr. Sean Evans reminded us that when BP is ultimately controlled, one might expect neurological recovery to occur within days to months, but rarely PRES is not actually reversible or there may be recurrent seizures.
Take Away Points:
PRES stands for posterior reversible encephalopathy syndrome, and is commonly associated with hypertensive emergency as well as pre-eclampsia/eclampsia as well as cytotoxic or immunosuppresive agents.
One might see white matter changes/ areas of edema on MRI specifically in the posterior or occipital regions of the brain but it is not present in all patients
Use IV labetalol or nicardipine as first line agents when treating HTN in PRES to lower the blood pressure carefully. Aim for 25% reduction in first 4 hours, as the blood pressure set point for hypotensive injury is much higher in patients with chronic hypertension!
Thanks again, Dr. Evans for your clinical pearls and to Dr. Jiang for a great case!
Today at the VA, we discussed one of the most common outpatient chief concerns (the 3rd most common, actually!): dizziness! Our patient, a 63 year old woman, had presented earlier that morning with a new and progressive sensation of spinning and double vision when looking in certain directions. We started out by distinguishing between the different reasons people might complain about dizziness, which broadly include: vertigo, presyncope, disequilibrium, and psychiatric (non-specific), all of which can be relatively well distinguished from one another by the patient’s history. When asking about dizziness, it is important to keep the question open-ended, as unprompted disclosure of a spinning sensation by the patient has a 95% PPV for vertigo, which drops to 75% if prompted by the provider! After characterizing our patient’s dizziness as vertigo, we then compared and contrasted peripheral and central vertigo by their respective distinguishing historical features as well as the most common associated diagnoses. Finally, we discussed that for our patient, the concern for central vertigo caused by CVA was very high (3 D’s of a posterior stroke: dizziness, diplopia, dysarthria), which was confirmed clinically by exam findings of a left internuclear ophthalmoplegia (INO) and right deviated uvula! Thanks to everyone who participated!
Take Home Points:
When a patient presents with dizziness, it is essential to let them describe the sensation in order to narrow you initial approach to the problem.
Remember the key features the distinguish peripheral and central vertigo (especially presence of headache and/or neurologic complaints) as most causes of central vertigo require urgent or emergent work-up.
The presence of INO is very suggestive of either: 1. MS or 2. brainstem stroke, so in conjunction with vertigo and diplopia, this exam finding made the diagnosis of a vertebrobasilar stroke (without MRI!).
Today our amazing 2nd year Med/Peds resident, Cathy Cichon, led us through a interesting case of a young woman brought in for subacute seizures and fever. We first walked through our protocol for acute management of seizures and status epilecticus. We then learned that the patient had been otherwise healthy with no significant history but had about recurrent seizures over the last 6 months. She then developed behavioral changes, paranoia and fevers in the last month, has since been more unresponsive and now has orofacial dyskinesia. We went over a thorough differential diagnosis for seizures, and the work-up we would like to do in this case. Ultimately this patient had a unremarkable work-up but based on her clinical history the neurology team had high suspicion for autoimmune, specifically anti-NMDA receptor encephalitis.
Our expert neurology discussant, Dr. Brenton Wright, helped us understand the pathophysiology for this rare condition and pointed out the often the gold standard test, the ant-NMDA receptor IgG autoantibody, that we test for will take weeks to come back. He noted it is therefore important once infection has been ruled out to start empiric treatment with steroids and IVIG or plasmapheresis. Patient may have a variable range of recovery but have better prognosis with earlier interventions. In about 50% of females patients with this condition they may have a pre-existing ovarian teratoma that may be leading to the formation of these autoantibodies.
Remember to treat acute seizures with IV ativan 2-4 mg, if recurrent can repeat dosing q5 min, max dose 0.1 mg/kg. If recurrent patient may also need loading with an AED such as fosphenytoin or valproate. If recurrent or continuous for 3 min, consider status epilepticus- these patients will need intubation and warrant ICU admission.
Consider getting testing for autoimmune conditions for young female patients with a clinical picture concerning for encephalitis. Consider specifically anti-NMDA receptor encephalitis for those presenting with seizures, acute psychiatric findings and orofacial dyskinesias.
Empiric treatment with steroids + IVIG or plasmapheresis, followed by rituximab, is often necessary for patients suspected of this condition, as confirmatory testing will be delayed. Remember to check for ovarian teratomas in female patients!
Thank you Dr. Cichon for a great case and to Dr. Wright for your expertise!
For our last neurology/emergency medicine Friday school session, our residents and interns tested their knowledge of neurology by working through several simulation sessions, courtesy of NCC expert Dr. Cynthia Gonzalez! We then learned about stroke syndromes and management! Check out the link below for some of the pearls from the sim sessions!
Today Dr. Barsky presented the case of a 47 year old male with sudden onset lower extremity weakness and a T10 sensory level. Imaging revealed extensive transverse myelitis with T2-T8 spinal cord involvement. These long spinal cord lesions suggest neuromyelitis optica, and serum aquaporin 4 antibodies are 99% specific for the diagnosis. Their sensitivity is only 50-70% however, and multiple sclerosis is still on the differential. Our VA teams competed to fill out illness scripts against the clock, with a win by teams 3 and 4! Thanks to our Neuro colleagues and Dr. Katie Longardner for helping build this challenge!