Shingles & Stroke – May be time to get that vaccine?

New research has shown that shingles is a risk factor for TIA and stroke.

This increased risk is independent of other stroke risk factors such as hypternsions, smoking and high cholesterol.

People under 40 years of age who had had shingles were 74% more likely to have a stroke than those who had not.

Shingles is caused by the varicella-zoster virus (chicknepox).  The infection lies dormant in nerve roots, but when reactivated leads to a painful skin rash followed in some cases devastating post herpetic neuralgia

Current recommendations from the US Centers for Disease Control and Prevention (CDC) are that anyone aged 60 years and older receives the herpes zoster vaccine.

The role for vaccination in younger individuals with vascular risk factors, and whether this can reduce stroke risk, has yet to be determined.

How could shingles cause stoke?  For cases where the rash involves the head and neck have led to virus spread into the cerebral arteries via the trigeminal nerve, leading to arthritis and stroke.  For other cases, the mechanism is unknown.

Click here to find out more about this study.

Why Oliver Sacks is One of the Great Modern Adventurers | Science | Smithsonian

Why Oliver Sacks is One of the Great Modern Adventurers | Science | Smithsonian.

The origin of the evil conformation of prion proteins – Medical News Today

The origin of the evil conformation of prion proteins – Medical News Today.

Plasma Exchange For Myasthenia Gravis

Posted by Christopher Orr,  Drexel University College of Medicine 2014

Ms. AB presented last week in the Neurology office with shortness of breath and weakness, and she knew it was from her myasthenia gravis.

She was already on an anticholinesterase inhibitor, but it was very apparent that she was suffering from a severe exacerbation of myasthenia gravis.

We sent her to the Emergency Room in order to be admitted so she could receive plasmapheresis in order to minimize the antibodies that were blocking the acetylcholine receptors at her neuromuscular junctions.

To give a brief history of Ms. AB’s myasthenia gravis, she was diagnosed in the Fall of 2013 when she presented with muscle weakness and difficulty breathing.  She was treated with plasmapheresis during that initial episode, and improved.

In the interim, she had also been given steroids to reduce the immune response of her autoantibodies towards her acetylcholine receptors, but this actually caused increased leg weakness, more likely from steroid myopathy than myasthenia gravis.

Unfortunately she experienced another exacerbation in December, and she was treated with intravenous immunoglobulin (IVIG).  What is interesting is that when she was treated with IVIG, her symptoms did not improve as they had done plasmapheresis.

There is limited research on the efficacy of IVIG in comparison to plasmapheresis in the literature.  A comparison study of IVIG vs. plasmapheresis waspublished by Mandaway et. al. in the Annals of Neurology in 2010 and included 1,606 patients – both therapies showed similar clinical outcomes in terms of both mortality and complications.  From a purely financial perspective, IVIG was more cost effective because of lowered length of stay and total inpatient charges.

However, a smaller study published by Stricker et. al. in JAMA in 1993 reported 4 patients who did not respond to initial IVIG treatment but later responded to plasmapheresis.  There were no definite prognostic factors mentioned that might explain why plasma exchange may be better than IVIG in certain patients.  The article stated further research was needed.

Ms. AB did present with myasthenia gravis at a later age of onset than is typically observed.  For future studies that compare IVIG to plasmapheresis, I would be highly interested in a subgroup analysis on a patient’s age and the efficacy of the 2 treatment modalities of IVIG and plasmapheresis.

When we saw Ms. AB in the hospital, she was already doing much better with plasmapheresis.  In addition, we were For the future, Ms. AB would likely be discharged to a rehabilitation facility and there are considerations to start her on CellCept (mycophenolate mofetil).  It would be preferential to start the patient on CellCept as an immunomodulatory drug to decrease the autoantibodies against her acetylcholine receptors and reduce her need for plasmapheresis.

I chose to write a reflection on Ms. AB for 2 reasons.  First, she and her husband are both very kind people, and it is a pleasure to see her improve.  Second, I love technology in medicine and healthcare.  When we saw Ms. AB’s plasmapheresis treatment, it was fascinating to see the apparatus that was using centrifugal force to spin her blood and separate her plasma from the WBCs, RBCs, and platelets.  The mechanism behind performing the plasmapheresis was to take off her plasma, which had the autoantibodies, and replace new plasma with albumin.  Please look below to see a picture of a plasmaphresis apparatus and an explanation of how it works.  After my experience seeing Ms. AB, it was a pleasure to treat her and learn from her condition.

Pictured above is an apparatus that is used for the plasmapheresis treatments.  Although this machine may seem very intimidating, it operates on the basis of how spinning blood can separate the blood into different components, such as the plasma, RBCs, WBCs, and platelets. To explain in a simple manner, a central venous line is obtained from the patient so blood can be brought to the machine and spun.  After the plasma is removed by the centrifugal force, the remaining components (RBCs, WBCs, and platelets) is added with albumin and saline (a protein found in plasma) then reintroduced into the patient.

In many instances, diseases can be complicated to comprehend.  I wanted to give a better understanding of myasthenia gravis.  I hope this picture and caption that I included make the disease easier to digest.

The above image shows the synapse of a neuromuscular junction.  In a healthy patient, the acetylcholine can bind the acetylcholine receptor and produce a response in the muscle.  In a patient with myasthenia gravis, the antibody (shown in green) is blocking the acetylcholine receptor and preventing the acetylcholine in the synapse from reaching the muscle.  This picture is a good educational tool because it also shows how the treatment of pyridostigmine (Mestinon) can improve a patient’s symptoms.  The acetylcholinesterase (AChE, the red pac-man figure) is what degrades the acetylcholine in the synapase.  Pyridostigmine is an anticholinesterase inhibitor and impedes the red pac-man figure in the picture from working.  Therefore, pyridostigmine increases the amount of acetylcholine in the synapse that can reach the receptor and will improve the symptoms in an episode of myasthenia gravis.

Duchenne Muscular Dystrophy

Posted by Elliot Dubowitch from Drexel University College of Medicine Class of 2014

Duchenne muscular dystrophy (DMD) is one of the four main groups  of muscular dystrophy, a muscle disorder that affects and weakens the musculoskeletal system.

Muscular dystrophies are genetically inherited and progressive.

DMD is inherited in an x-linked manner.  This means that the mother, who is unaffected, is a carrier for the disease and has a 50% chance of passing it on to her male children.

The disease is caused by a deficiency in the Dystrophin protein, a complex that anchors the muscle to surrounding tissue.

This disease has a wide spectrum of symptom severity, depending on the type of genetic mutation, with Duchenne being very severe, and Becker’s muscular dystrophy being much more mild.

The symptoms in DMD are not usually present at birth.  As the child ages, however, they symptoms will gradually become worse and worse.  Most children are unable to walk by thirteen years of age and die in their twenties due to respiratory failure.

One of the earliest signs of DMD is called to as the “Gower Maneuver.”  Although not pathognomonic for DMD, this maneuver is a sign for proximal muscle weakness and is often correlated with DMD.  Below is a clip of a child performing to Gower’s maneuver to stand.  The patient must “walk” up his body using his hands from a sitting position due to weakness in his hip and thigh muscles.  Below is a video clip demonstrating this.

Another early sign is calf pseudohypertrophy.  Although the muscle looks bigger, it is not necessarily stronger, as the functional muscle is replaced by nonfunctional fibrous tissue.

Unfortunately, there is currently no cure for DMD.  However, there is symptomatic treatment available, such as respiratory support, cardiovascular monitoring and treatment and (if needed) surgery for scoliosis.

Steroids are the only current medication that has been shown to keep the boys walking longer.  A study was conducted in which one group of boys were given steroids daily, whereas the other group of boys were given steroids 10 days on and 10 days off.  The boys receiving daily steroids walked on average until the age of 14.5 year, while the boys receiving steroids intermittently walked to only 12 years of age.  The boys receiving continuous steroids also had more side effects including weight gain, mood swings, increase risk of infection, and other side effects of steroid usage.  If one is to consider steroid use, it is imperative to remember that it must be used at the time the child is still ambulating.  The boy will not regain lost function, however he may retain his current function longer.  In the future we hope that new drugs like VBP-15 will hopefully provide the benefits of corticosteroids without some of the side effects.

Genetic research is currently being done to hopefully find a cure for this disease.

MMC’s Stroke Program Recertified & Don’t Forget Our Stroke Support Group.

Monmouth and HealthSouth’s Stroke Survivors Support Group

Where: The Cafeteria at The Rehabilitation Hospital of Tinton Falls

When: First and Third Wednesdays Each Month

Meetings are led by Stroke Survivor Dr Zaback.

Contact Shirley at 732-460-6742 for more details.

CJD – A downward spiral of depression into dementia and death

Post prepared by Dr Mariam Kemal, PGY-3 (Internal medicine), Monmouth Medical Center

Case History:

This 66 year old female had been living alone independently.  However, her neighbors became concerned when she had seemed more withdrawn than usual for about a month, and then stopped going out of the house and paying her utility bills.  Ultimately, one of them  noticed a dead cat in the house, and immediately called patient’s son who lived out-of- state, and he requested that she be admitted hospital.  At the time of her initial evaluation, she was depressed and had a urinary tract infection.  She was treated for the infection, and when she expressed suicidal ideation she was transferred to the psychiatric unit. While she was on the psychiatric unit she developed slurred speech, right arm clumsiness and and unsteady gait.   She was transferred back to the medical service and underwent a diagnostic evaluation.   Her brain MRI showed diffusion restriction in left putamen and caudate nucleus. Her EEG was also abnormal.  Her spinal fluid was ultimately positive for presence of Protein 14-3-3, indicating Creutzfeldt –Jacob disease.  She has progressed to a very debilitated state in just two week – Her speech was limited to a few intermittent slurred words, she was not able to walk and had diffuse myoclonic jerks. She was transferred to hospice.  Her brain was sent for autopsy to The National Prion Disease Pathology Surveillance Center which confirmed the presence of abnormal protease resistant prion protein (PrPSc), commonly identified as PrP 27-30, confirming the diagnosis of sporadic Creutzfeldt-Jacob disease.
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What is Creutzfeldt-Jakob disease?

Creutzfeldt-Jakob disease (“CJD”) is a rare brain disorder that causes rapidly progressive dementia with muscle twitching, leading to death within several months.

CJD usually affects older adults.

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It is caused by abnormal proteins called “prions” that infect the brain.

“Classic” CJD has been transmitted by infected organs during transplant surgery.

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“Variant” CJD (“mad cow disease”) has been transmitted by infected beef.

In addition to dementia and myoclonus, many CJD patients also exhibit behavioral change (including depression), balance problems, and sleep disturbance.

It is the presence of these unusual clinical features, and the rapid rate of clinical deterioration, that distinguish CJD from other dementias like Alzheimer’s disease.

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How is Creutzfeldt-Jakob disease diagnosed?

MRI imaging of the brain can show characteristic findings:

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The electroencephalogram (EEG) can show periodic complexes:

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The spinal fluid can show the 14-3-3 protein.

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However, a brain biopsy demonstrating spongiform change is still necessary to confirm the diagnosis in many cases:

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How is Creutzfeldt-Jakob disease treated?

Sadly, there are no treatments that can stop or cure the disease, and all affected patients die within several months.

2014, 100th Anniversary for Anosagnosia

Anosagnosia

Here’s an illustrative example from a conversation with FD, an elderly woman who had had a right hemispheric stroke one week before, leaving her paralyzed on the left side and confined to a wheelchair:

How are you feeling today?

FD: I’ve got a headache.  You know, doctor, I’ve had a stroke so they brought me to the hospital.

Can you walk?

FD: Yes (FD had been in a wheelchair for the past week, and cannot walk)

Mrs D, hold out your hands.  Can you move your hands?

FD: Yes

Can you use your right hand?

FD: Yes

Can you use your left hand?

FD: Yes

Are both hands equally strong?

FD: Yes, of course they are equally strong.

Mrs D, point to me with your left hand.

FD: (Her hand lays paralyzed in front of her).

Mrs D, are you pointing to my nose?

FD: Yes

Can you see it pointing?

FD: Yes, it is about 2 inches from your nose.

Mrs D, can you clap?

FD: Yes, of course I can clap.

Mrs D, will you clap for me?

FD: (She proceeded to make clapping movements with her right hand, as if clapping with an imaginary hand near the midline)

Are you clapping?

FD: Yes, I am clapping.

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The term anosagnosia was first used by Joseph Babinksi on June 11, 1914 in a brief communication presented to the Neurological Society of Paris.

He described two patients with left hemiplegia who didn’t know they were paralyzed.  The word comes from the Greek words nosos, “disease”, and gnosis, “knowledge”.

Affected patients deny their deficit, and overestimate their abilities, they state that they are capable of moving their paralyzed limb and that they are not different than normal people.

Their false belief of normality persists despite logical arguments and contradictory evidence – they may even produce bizarre explanations to defend their convictions.

If they admit any impairments, they will attribute them to other causes (i.e. arthritis, tiredness, etc.).

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But, this syndrome is not only seen with hemiplegia.

Visual anosognosia or Anton-Babinski syndrome is a rare neurological condition related to cortical blindness.  Affected patients deny their blindness and affirm adamantly that they are capable of seeing. The clinical presentation includes confabulations – instead of admitting blindness, they will make up answers when asked about what they see.

Mr Magoo is a great TV example of Anton’s syndrome – unaware of his loss of vision, he misinterprets and confabulates his way into trouble.

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Anosognosia may occur as part of receptive or Wernicke’s aphasia – affected patients cannot monitor and correct their own speech  errors and may appear angry and frustrated when the person they are speaking to fails to understand them.

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Watch this lecture, by Dr V. S. Ramachandran for more information on this fascinating syndrome: