Cortical Ribbon 5.0

Welcome to the fifth edition of the Cortical Ribbon. We are especially excited about this edition as it coincides with our official blog debut! We welcome you to subscribe to our blog at the bottom of the website, but no worries, we will sign you up if you forget.

Case of the Week: Strokes, Seizures, Cavernomas, Oh My!

.. So, JAR, what do you see on the head CT? Well Jim, it certainly looks abnormal..

An elderly right handed male of Latin American origin with a history of epilepsy and remote prostate cancer presents to the emergency department for acute onset of mild aphasia, noticed by family upon waking up in the morning approximately 6 hours ago. A stroke alert is called and the Jar evaluates the patient, noting this head CT:

CR5 Cavernoma CT

One clear finding is the hypodensity in the left lateral frontal lobe concerning for a new infarct, which could explain the patient’s symptoms. Patient was clearly out of the window for tPA and thrombectomy was not pursued given mild symptoms, but what is that other finding? Metastases? Abscess? If he was in the window, would he get tPA? In this patient, definitely not. With additional history from the patient and the chart, this was already known to be a cavernoma. On additional CT slices, similar lesions are scattered throughout both the supra and infratentorial regions of the brain. Cavernomas can look like a multitude of other pathologies on CT scan, therefore MRI is the study of choice.

On MRI, cavernomas are described as a classic “popcorn” or “berry” appearance (but, really, do we actually think they look like that? Just saying…) As most cavernomas bleed intermittently, T1 and T2 can show varying degrees of signal based on ages of blood products. GRE, as shown in the MRI images above, clearly show the abundance of cavernomas in this particular patient. While not confirmed with genetic testing, this patient most likely has a familial cavernoma syndrome given the sheer number of cavernomas found. Interestingly, nearly all cases of familial cavernoma syndrome in Hispanic individuals are linked to a mutation in CCM1 (or KRIT1) on chromosome 7q whereas non-Hispanic cases are linked to CCM2 and CCM3 (or PDCD10). It is not totally understood what these genes do, but they are generally thought to help stabilize the interaction between adjacent cells within a blood vessel. Disruptions of these genes result in leaky vasculature and the development of cavernomas.

It is worth mentioning that most patients with cavernomas have the sporadic form, which presents usually as a single lesion. Familial forms tend to have multiple lesions and are inherited in an autosomal dominant pattern. In either case, presentations range from seizures (our patient had resultant epilepsy), focal neurologic deficits, hemorrhages or headaches. This patient unfortunately presented to us with an ischemic stroke, potentially from underlying atherosclerotic disease and likely not related to his cavernomas (talk about Hickam’s dictum.) Cardionet was not pursued as patient would not be able to start anticoagulation. However, a risk benefit conversation was had for stroke reduction and the patient was discharged on a daily aspirin.


Pediatric Pearls: Acute Disseminated Encephalomyelitis (ADEM)

Adam is a regular, healthy 7 year old boy who presents with multifocal neurologic deficits, increasing behavior problems, irritability, and confusion following a recent febrile viral illness. MRI reveals extensive white matter changes. Could Adam have ADEM?

Let’s just break it down real simple. ADEM is an acute, demyelinating event associated with encephalopathy, multiple neurologic deficits and diffuse, asymmetric white matter (or deep gray matter) changes on MRI. Monophasic ADEM should happen only once, but can fluctuate for up to three months. If it persists beyond that or new symptoms develop, it is then considered multiphasic ADEM. Any more than that leads you to think about multiple sclerosis as the diagnosis instead. Differentiating the two at onset can be difficult, but the encephalopathy is the key. ADEM requires it, MS usually lacks it. Additionally, ADEM typically occurs at younger median age (7 years old) compared to pediatric MS which occurs at a median age of 12 years old. ADEM is classically monophasic, whereas MS is characterized by multiple demyelinating events separated in space and time.  Brain MRI findings in MS are often periventricular, smaller, and more demarcated whereas in ADEM, lesions typically are larger, bilateral, spare the periventricular area and have indistinct borders; they may also involve the deep gray matter structures. Lastly, CSF panel reveals more pleocytosis in ADEM compared to multiple sclerosis. Thankfully, treatment is rather similar and involves high dose IV steroids (20-30 mg/kg of methylprednisolone) for 3-5 days. Gotta love dosing by weight. If there are ongoing symptoms you can initiate an oral steroid taper over 10-14 days.  If symptoms do not improve, 2nd line treatment is with IVIG (2g/kg divided over 2-5 days). If very severe, ADEM can also be treated with plasmapheresis. Prognosis is generally favorable, although 11-17% of children may remain with residual motor deficits.


The Price is Rite Review

All that Shakes Isn’t A Seizure – Lance-Adams Syndrome

Armstrong Ullrich

Individuals who experience out of hospital cardiac arrest have an approximately 10% chance of surviving. While intensivists have used the presence of acute spontaneous myoclonus as a prognostic indicator in the immediate post-anoxic period, there is an additional sort of shakes to watch for in the recovery period. It is the syndrome currently known as Lance-Adams Syndrome. Described in the era of eponyms, the disorder is characterized by a persistent intention/stimulus induced myoclonus after regaining consciousness and does not always correlate with EEG abnormalities (but can be associated with vertex spike-wave complexes).  It can develop days or possibly weeks after the anoxic event (and thus may first poke its shaky little head at rehab). While uncommon (<200 described cases) it has been brought up three times in the past week by fellow neurologists – the perks and perils of tertiary care training. Treatments vary given the rare nature of the disorder, but clonazepam or AEDs such as levetiracetam may be helpful in controlling these movements.


This is your Brain on Poetry – Weekly Neurology Haikus

(Daily Double Edition)

Curbside, Oh Curbside
Recommendations by Phone
Let me stay asleep!

Hypothalamus
You make me hot and hungry
Can you help me sleep?

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