Posts about neurology from 2 of our recent medical students

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Posted by Ilya Grinberg:

Last week I participated in the MDA clinic and there was one patient who really stuck out in my mind. He was a 17 year old male who had Duchenne Muscular Dystrophy. He was completely wheelchair-bound and could barely move any of his extremities. This obviously meant that he was completely reliant on people to take care of him and that he could not participate in the day to day activities that a normal person could. He was also starting to have breathing difficulty and probably would need to start therapy on a ventilator soon too.

DMD is an x-linked recessive disorder that affects boys from an early age. It affects 1 out of 3600 boys so it is not too uncommon. The disease is due to a mutation of the structural protein Dystrophin which is normally found in all muscles. The protein is found on the X chromosome which explains why it is an X-linked disease. Patients are usually born normally but will start gradually developing symptoms during the first few years of life. The first observations are usually progressive muscle weakness in proximal muscles in addition to muscle atrophy. One of the key clinical signs in DMD is calf psuedohypertrophy which occurs due to fibrosis of the necrotic muscle tissue. Another common finding that presents early on is Gower’s sign, where the patient uses his arms to stand up due to weakness in the proximal leg muscles.

Click here to find out more about DMD.

 

Posted by John Soliman:

What is Neurology one may ask? Prior to and during medical school I feel like the exposure to the study of neurology was very limited. Interaction between neurological patients and medical professions was far and few between. I have had little encounters with the realm that lies ahead. Prior to starting the clerkship I was very intimidated due to my lack of knowledge and ignorance. I can say jokingly I barely knew how to spell Neurology. Even the basics of neurology such as anatomy was daunting usually getting hung up on learning one part or area as seen in this video. I have to say I had something in common with Pinky.

After the 4 weeks of neurology I have realized that neurology covers a broad realm of knowledge and information on the central and peripheral nervous system.

During my clerkship I was lucky enough to be exposed to many patients encountering a lot of this medical conditions and problems. I was able to identify and correlate symptoms with disease states and vice versa.

The most memorable experience was the MDA clinic. I was able to meet and was integrated into the care of a lot of the Myotonic dystrophy patients. I was able to see hear their day to day life experience, talk to their care givers and be able to help with their care. After seeing patients like this it really brought “problems” Into perspective. The amazing thing was the broad range of how these medical conditions affect each individual differently.

I had the opportunity to see two brothers who are both affected with Myotonic Dystrophy. One brother can walk, talk and act normal with minimal weakness while the other was wheel chair bound. Talking to them brought home how a muscular dystrophy can affect the lives of affected individuals. Myotonic dystrophy is an autosomal dominant genetic process which means it can affect 50% of the carriers offspring. This may affect a family’s decision on having kids both mentally and psychologically. In this specific case, one of the brothers and his wife decided to adopt children due to the high risk of having a child with myotonic dystrophy. Dealing with something like this is a full time job on its own so it can be draining mentally, physically and financially on families.

Overall the experience has been great as I have gotten to see patients with medical conditions that I may not be able to see again. This clerkship has been knowledgeable and I has encountered a broad real of neurology that I never had experienced in the past.

Click here to find out more about Myotonic Dystrophy.

Click here to find out more about MDA Clinic at Monmouth.

George Huntington, On Chorea

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George Huntington (1850-1916) was a medical practitioner in Dutchess County New York.

His father and grandfather had both been doctors, and his family had lived on Long Island since 1797.

He gave his classic presentation, “On Chorea”, at the Meiga and Mason Academy of Medicine in Middleport Ohio in 1972, and this was subsequently published in the Medical and Surgical Reporter of Philadelphia on April 13, 1872.

This took place just one year after he graduated from his medical training at Columbia University.

He later explained his interest in the condition that now bears his name:

Over 50 years ago, in riding with my father on his rounds, I saw my first case of the “disorder”, which is the way the natives always referred to the dreaded disease.  I recall it as vividly as though it had just occurred but yesterday.  It made a most enduring impression on my boyish mind, an impression which was the very first impulse to my choosing chorea as my virgin contribution to the medical lore.  Driving with my father through a wooded road leading from East Hampton to Amagansett we suddenly came upon two women both bowing, twisting, grimacing.  I stared in wonderment, almost in fear.  What could it mean?  My father paused to speak with them and we passed on.  Then my Gamaliel-like instruction began; my medical instruction had its inception.  From this point on my interest in the disease has never wholly ceased.

Huntington’s disease (HD) is now know to be caused by a genetic mutation.  It is inherited in an autosomal dominant fashion, so that any child of an affected person typically has a 50% chance of inheriting the disease.  The mutation is a triplet repeat, so the genetics of HD lead to anticipation, a phenomenon whereby the disease may begin earlier and more severely in each successive generation.

HD can present at any age, but most often begins around 35-44 years with psychiatric disturbance such as depression or forgetfulness.  Neurological manifestations such as unsteady gait and the  jerky body movements (chorea) noted by George Huntington come later, followed by a progressive dementia.

Genetic testing is available, but pre-symptomatic testing for family members, who are at increased risk for the disease, is controversial since there is no treatment for HD.

Find out more about HD from the Huntington’s Disease Society of America.

Recent study links marijuana use to structural brain changes

 

 

Post  prepared by Amanda Baker, Drexel University College of Medicine Class of 2014

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A study recently published in the Journal of Neuroscience by Harvard researchers have linked casual marijuana use to structural changes in distinct areas of the brain.

These areas, the nucleus accumbens and amygdala, and are largely involved in recognition of reward, motivation, fear, and memory.  In this study, the brain scans of 20 young adult casual marijuana users were compared to those of 20 young adult non-users.

While the results clearly demonstrated significant structural differences between the two groups, the structural changes have not been correlated with consequences in mental or physical functioning.  In other words, researchers aren’t entirely sure of the impact of these brain changes.

 

Casual marijuana use may damage your brain

The debate regarding the use of marijuana medically and recreationally is ongoing in the United States.

Although the Drug Enforcement Administration(DEA) categorizes this drug as Schedule I, “with currently no accepted medical use and a high potential for abuse”, many argue that there is, in fact, much benefit to medical marijuana, especially in comparison to other sedating pain medications.

This is reflected in 21 state laws which have legalized medical marijuana to varying degrees.

Last summer, Dr. Sanjay Gupta completed a documentary “Weed” highlighting the benefit of medical marijuana:

However, some states such as Washington and Colorado, have gone one step further by legalizing marijuana for both recreational and medical use.

Given the ongoing research on the effects of marijuana, perhaps this new study will call into question continued legalization of the most widely used recreational drug in America.

 

Click here to find out more.

 

Gamma knife tremor patient comes back to get the other side done!

Our first tremor patient treated with gamma knife radiosurgery recently came back, one year later, to have the other side treated.
He has essential tremor, which was affecting both arms.
He had undergone treatment to the left brain for right sided tremor last year.
He was so pleased with his results, he recently came to have the right brain treated to address the left sided tremor.
Here is his most recent video.
Note the action and postural tremor on the left (untreated) side, and the fact that he has almost not residual tremor on the right (treated) side.

Click here to find out more about Gamma knife radiosurgery for tremor at the Monmouth Neuroscience Institute.

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.

PLEDS

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.

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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.