Limbic Encephalitis

Post prepared by Precious Ramirez-Arao, Monmouth Medical Center PGY3

A 60 year-old female was found lethargic lying in a pool of feces by roommate.

EMS was called and was immediately brought to the hospital.

In the emergency department she had a witnessed generalized tonic-clonic seizure.

Her roommate relates she had episodes of confusion and short-term memory loss over the past few weeks.

She remained lethargic over the next 72 hours in the hospital.

48-hour EEG monitoring showed diffuse 2 to 3 Hz delta slowing with periodic lateralized epileptiform discharges emanating from the left frontal temporal region.

T2 weighted image of the brain showed signal abnormality of the left mesial temporal lobe and the pulvinar with diffusion restriction in the left hippocampus consistent with limbic encephalitis.

Limbic encephalitis (LE) is a subacute syndrome of seizures, personality change and cognitive dysfunction, typically evolving over days to weeks.

Autoimmune and paraneoplastic forms have been described. The most common neoplasms associated with paraneoplastic LE are lung cancer (usually small cell), thymoma, ovarian or testicular teratoma, breast cancer and Hodgkin lymphoma. The associated autoantibody depends on the tumor type. Lung cancer and thymomas are associated with anti-VGKC while ovarian or testicular teratomas are associated with antiNMDA antibodies.

Neurologic symptoms can precede oncologic diagnosis for several months to years and initial CT scans are typically unrevealing.

Nevertheless, prompt and thorough evaluation for malignancy including PET and CT scan of the chest, abdomen and pelvis should be initiated. Symptomatic treatment includes corticosteroids, plasmapharesis and intravenous immune globulin.

 

The Neurology of JFK’s Assassination

As a devout conspiracy theorist, I could not resist posting about the John F. Kennedy assassination during its 50th anniversary.

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This story contains at least 3 neurological issues of interest.

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First, there’s the head wound, and what it tells us about the location of the shooter:

Conspiracy theorists have stressed witness testimony that the back of Kennedy’s head was blown out, suggesting a shot from the Grassy Knoll.

Lone assassin theorists have stressed the photographic evidence and the autopsy x-rays, which show the back of the head intact. The panel (inserted above) shows four of the dozens of versions of Kennedy’s head wound.

Click here to find out more.

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Second, even if you believe Lee Harvey Oswald was the lone assassin, some conspiracy theorists would have you believe he was a Manchurian Candidate, manipulated by mind-control experts to carry out the assassination of JFK.

These theories are supported by what is known about the CIA’s experiments with mind control in the 1950s, and Oswald’s alleged CIA connections.

Click here to read more about this.

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Finally, there is Jack Ruby‘s murder of Lee Harvey Oswald:

The matter of neurologic interest here is that Ruby’s defense at trial was based on an abnormal electroencephalograph.

Defense expert Frederick Gibbs, one of the pioneers in the use of electroencephalography for the diagnosis and management of epilepsy,  testified that Ruby’s EEG showed right temporal 6/s sharp waves, and that this was evidence of “psychomotor epilepsy”.

Gibbs suggested that affected patients manifest personality instability, lack of emotional control, convulsive and excessive types of behavior.  He, and other physician experts at the trial further postulated that Ruby killed Oswald during a fugue state induced by a psychomotor seizure.

This image of Ruby shooting Oswald was used at trial – Ruby is seen using his middle finger to pull the trigger, with his left hand thrown out in the opposite direction, supposedly indicating a seizure.

The prosecution’s neurology expert disagreed, stating that the EEG findings were a “slight abnormality” and didn’t indicate epilepsy.  Furthermore, he indicated that Ruby’s demeanor and behavior, as described by witnesses, were not consistent with a psychomotor seizure.

Ruby was convicted, and sentenced to death.

Click here to find out more about the neurology at the trial.

This EEG “finding” is now known to be a normal variant with no clinical significance.

This case underscores the importance of treating the patient, not the test result, an adage well known to true clinicians everywhere.

Brain-to-Brain Interfacing: Next Step… Mind Control

Post written by Anne Verlangieri, MS IV at Drexel University College of Medicine, Class of 2014

Step aside Obi-Wan Kenobi, this is not the mind control you’re looking for. A recent study conducted by researchers at Harvard University has demonstrated a method for non-invasive, functional linkage of brain activity from human volunteers and Sprague-Dawley rats. The method, dubbed brain-to-brain interface (BBI), utilizes electroencephalographic steady-state-visual-evoked potentials (SSVEP) and transcranial focused ultrasound (FUS). The goal of the process is to allow human volunteers to use visual stimuli to elicit motor responses from the tail’s of rats. To understand how BBI works, we’ll need some background on the SSVEP and FUS segments.

SSVEP

Numerous neurophysiological studies have shown that there is increased neural activity elicited by a visual stimulus with directed attention. In other words, observer attention on a specific visual stimuli is more important that the stimulus itself, in producing measurable spikes in neural activity. SSVEP utilizes EEG based brain-to-computer interfacing (BCI), to take advantage of this idea. In practice, a human volunteer is equipped with an EEG and instructed to gaze on a designated visual stimulus. The EEG reads the pre-synchronized neural activity, linking the volunteer’s brain with an SSVEP computer.

FUS

Transcanial focused ultrasound has been used clinically as a non-invasive therapy for certain brain disorders, (ex. Deep brain stimulation in Parkinson’s Disease) as well as thermal ablation of brain tumors. This technology allows for region-specific brain stimulation, and when set at low acoustic energy, has been shown to excite or suppress rabbit motor/visual cortices; effectively creating a computer-to-brain interface (CBI).

How BBI works

(Figure 1. The schematics of the implemented brain-to-brain interface (BBI). The implementation consists of steady-state visual evoked potential (SSVEP)-based brain-to-computer interface (BCI: on the left column) and focused ultrasound (FUS)-based computer-to-brain interface (CBI) segments (on the right column). [doi:10.1371/journal.pone.0060410.g001]

The set-up shown in figure 1, demonstrates how SSVEP and FUS are utilized to link the human volunteer and with the rat’s brain. The volunteer is instructed to look at a specific visual target, creating an increase in EEG bandwidth corresponding to that specific visual stimulation frequency. A SSVEP detector reads the increase in EEG bandwidth and triggers the activation’s of the FUS, which stimulates specific motor area’s of the rats brain, resulting in a twitch in the rat’s tail. Here’s the experiment in action:

What can we do with this technology?

Certainly, the possible applications of BBI are far reaching. The studies authors proposed that this technology could one day be used for indirect sensory/somatomotor communication allowing an increased degree of understanding during verbal communications between speaker and listener.
Other’s have suggested “Hive mind” like problem solving, via a linked think-tank. And of course there is the potential for further human-to-animal interaction; pet owners would jump at the chance to know just what Fido is thinking.
Another recent study used this technology for one student to control another student playing a video game at a remote location.
Regardless of the application, it will be important to look at the legal, ethical, and privacy concerns involving technology that has the potential to transmit thought from one individual to another. The study is free to download and read here.

Epilepsy surgery and functional MRI

Epilepsy surgery is an option for patients with intractable partial onset seizures that are not controlled by oral medications.  Epilepsy monitoring is used to localize the seizure focus, often a lesion or abnormal area of brain located in the temporal lobe.  That part of the brain is then carefully removed to prevent future seizures:

A patient with a brain abnormality in the R temporal lobe (top) undergoes brain surgery to remove that area of brain and prevent future seizures.

Epilepsy surgery is very effective and yet still underutilized for treating seizures.

Left temporal lobe resections are more risky that right-sided cases, because the left hemisphere controls language functions in most (even left handed) patients.  Surgeons have to be very careful planning seizure surgery on the left side to be sure that they do not damage brain critical for speech and language and leave the patient with aphasia.

That’s where functional magnetic resonance imaging (fMRI) comes in.  fMRI goes beyond the conventional imaging of brain structure, and can actually localize regional brain functions by detecting changes in regional blood flow in response actual or imagined activity.

fMRI is increasingly being used to evaluate candidates for epilepsy surgery by identifying important functional regions within the brain, including unpredictable patterns of functional reorganization, to prevent unexpected post-operative deficits.  Click here for a link to a paper with illustrative cases.

Monmouth’s New Onset Seizure Center Opens in June!

New onset seizures can be isolated events or the harbinger of future epilepsy.

Decisions about starting medications and restricting driving are complicated, and are best made by neurology sub-specialists (“epileptologists“) after a detailed evaluation that usually includes an electroencephalogram (EEG) and brain magnetic imaging study (MRI).

Monmouth Neuroscience Institute is pleased to announce the opening of the region’s first New Onset Seizure Center in June 2013.

Patients who come to the emergency room with their first seizure can be stabilized and then sent home with instructions to follow-up in New Onset Seizure Center, an integral part our Certified Epilepsy Center within one week.

All patients coming to the center they will undergo an EEG, MRI of the brain and a visit with one of our board certified fellowship-trained epilepsy experts during a single visit.

This avoids hospitalization and hasty decisions about medical management.

Click here to find out more about the center.

Monmouth Epilepsy Program Receives NAEC Certification

Monmouth Medical Center’s Epilepsy Program was awarded prestigious level 3 certification today by the National Association of Epilepsy Centers!

The National Association of Epilepsy Centers (NAEC) is a non-profit  association with the primary objective of connecting people with epilepsy to specialized epilepsy care and epilepsy centers.

Founded in 1987 by physician leaders committed to setting a national agenda for quality epilepsy care, the NAEC educates public and private policymakers and regulators about appropriate patient care standards, reimbursement and medical services policies.

NAEC works in conjunction with existing scientific and charitable epilepsy organizations.

A third-level center must provide all the medical, neuropsychological, and psychosocial services needed to treat patients with refractory epilepsy to achieve certification.

Click here to find out more about Monmouth’s Epilepsy Program.

Click here to find out more about seizures and epilepsy.

American Academy of Neurology Identifies 5 Questionable Practices

It’s no secret that health care costs are escalating.

Although physicians value their independence, part of the problem here is the unregulated use of costly tests, medications and procedures, many of which are unproven and of questionable benefit.

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Evidence based medicine assess the strength of the evidence of risks and benefits of treatments (including lack of treatment) and diagnostic tests in an attempt to help clinicians predict whether a treatment will do more good than harm.

However, less than 1 in 5 medical decisions in the USA are made based on evidence-based medicine.

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The American Academy of Neurology has recently taken a step in the right direction by publishing a list of 5 costly neurology practices which continue to be widely performed despite being of little proven benefit:

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1. Don’t perform electroencephalography (EEG) for headaches.

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2. Don’t perform imaging of the carotid arteries for simple syncope without other neurologic symptoms.

Syncope is caused by global cerebral hypoperfusion, not carotid artery disease, so even if a carotid stenosis is identified, it would be asymptomatic (see point number 5)

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3. Don’t use opioid or butalbital treatment for migraine except as a last resort.

Butalbital is effective short term treatment for migraine, but commonly leads to rebound, which ultimately exacerbates migraine.  Click here for more information about migraine.

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4. Don’t prescribe interferon-β or glatiramer acetate to patients with disability from progressive, nonrelapsing forms of MS.

Interferon-β and glatiramer acetate, are effective for  relapsing phases of MS, but do not prevent the development of permanent disability in progressive forms of multiple sclerosis.  These medications cost more than $40,000 a year, and put a financial strain on the patients and their families to make the co-pays.  In some series as many as 10%-15% MS patients have progressive forms of the disease and yet are still taking one of these medications.

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5. Don’t recommend carotid endarterectomy (CEA) for all patients with asymptomatic carotid stenosis.

Surgery is of much less benefit for asymptomatic carotid stenosis, and should be reserved for those with a perioperative complication risk of less than 3% and a life expectancy of greater than 3 to 5 years. Click here for more information about surgery for carotid artery disease.

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Click here for more details.