An 83 y/o man was out for a daily walk in his neighborhood when he was discovered by a passerby to be face-down bleeding from his nose.  He was rushed to the local ED where he was confused and complaining of pain at multiple sites.  After extensive tests, the only pertinent abnormality was a small bleed in the lateral ventricle of the right frontal lobe of his brain.  Once returned to home and healed from his minor bruises and strains, it was clear to his family that there had been a change to this patient both physically and emotionally.  He no longer walked confidently and now exhibited small cautious steps.  For the first time in his life, he began having problems with urinary urgency in which he would have to hurry to the bathroom in order to urinate.   At night he began wearing adult diapers.  He also became more confused and had to be looked after by his wife.  In essence, he regressed to being more like a toddler.  His wife had to assume more of the daily household activities.   He stopped driving.  

On exam, the most striking feature was a slow, cautious gait with difficulty turning around.  Otherwise the exam was non-focal. 

First, an MRI of the brain showed signs that cerebrospinal fluid (CSF) was pushing into the adjacent brain (‘transependymal flow’).  In addition, when comparing the size of the ventricles (the chambers found in the center of the brain that house the CSF) to the size of the ventricles from a pre-accident MRI, it was clear that the ventricles were much larger.   This suggested that he had acquired hydrocephalus (‘water on the brain’). 

I then sent him for a lumbar puncture which revealed a normal pressure when performed – rather than an elevated pressure as one might expect.  I then performed a nuclear study called an Indium-111 cisternogram in which radioactive Indium-111 is injected into the CSF around the lumbar spinal cord.  In a normal person, this Indium-111 will not collect inside the ventricles but with patients who have a condition called Normal Pressure Hydrocephalus (NPH), the Indium-111 does end up inside the lateral ventricles.  This test was positive for this patient.

 He underwent placement of a ventriculoperitoneal (VP) shunt and immediately showed a return to his normal walking pattern and had resolution of his urinary urgency.  The confusion improved as well.  

DIAGNOSIS: NPH

Normal Pressure Hydrocephalus is thought to occur as a result of blockage of arachnoid granulations by degraded blood products.   Normally, the arachnoid granulations are responsible for resorption of the used CSF.  If an individual has experienced an intracranial bleed then they are theoretically at risk for developing NPH afterwards.  The classic clinical triad is ‘wild, wet and wobbly’ meaning worsening cognition, change in urinary habits and change in gait.  The treatment is placement of a VP shunt which will drain a small amount of the CSF continuously into the person’s gut.  

FACTS

Open enrollment ended on March 31st, 2014.  It will open again on October 1st, 2014.  It takes approximately 6 weeks for the insurance to be processed before the account becomes active.   You can enroll online (www.wahealthplanfinder.org) or go into a local public health department for personal help.   The one exception is individuals who make less than $15,000 a year.  These individuals can enroll anytime of the year.  

FACTS

The penalty for not having health insurance in 2014 is $95 per person up to $285 for a family (or 1% of a family income – whichever is greater).  However, by 2016, the penalty will increase so that for a family it will cost $2,085 (or 2.5% of family income – whichever is greater).

This is a 32 y/o man who two weeks prior experienced the painless onset of tingling of both feet extending up to his calves.  He had no weakness.   One week later, he noticed that each time he hyperextended his neck there was a tingling pain that would shoot around from his back to his nipples and this would go away if he returned his neck to a normal position.  Four days later, he began having difficulty initiating a stream of urine and by this time his balance had deteriorated to the point that he was forced to clutch onto furniture just to walk in his condo.  On examination, he had weakness for hand grip of the right hand, inability to feel a vibrating tuning fork on the toes, loss of pin sensation all the way up to a point just between his shoulder blades (in the center of his back) and a wide base to his stance as he was very unsteady.    

The MRI of the cervical spine demonstrates congenital narrowing of the spinal canal.  In addition, there are relatively small disc bulges and protrusions at several levels which in combination with the congenital spinal stenosis results in injury of the cervical spinal cord.  

DIAGNOSIS: Acute Cervical Cord Compression from Cervical Spondylosis 

This is a 68 y/o woman who for the prior four years showed a gradual worsening in her thinking that was evident to family members.  She had a change in her personality in which she became more disinhibited and careless.  For example, she began gambling at the casinos on a more regular basis losing up to $300 a month.  When her husband would confront her about her continual losses, she would respond, “Oh well – at least I’m having fun!”  Recently, her speech had grown slurred and she was having unexplained falls.  On exam, she demonstrated a wide-eyed expression on her face and could not move her eyes completely upward or downward.  She had a very rigid neck and there were brisk reflexes throughout all four limbs.  She had a tremor of her hands when she outstretched them in front of her body.  She could not draw the face of a clock correctly.

PET CT 

DIAGNOSIS:  Progressive Supranuclear Palsy (PSP)

Progressive Supranuclear Palsy (PSP) is diagnosed in 30 of 100,000 individuals each year.  For every 100 patients with Parkinson’s Disease, there is 1 person with PSP. 

PSP typically first presents during the 6th decade of life with problems walking.  Individuals will complain of unexplainable falls or stiffness often falling backwards instead of forwards.  The individual with PSP often develops insomnia as a part of the disease.  A small percentage will develop a rest tremor and be misdiagnosed as having Parkinson’s disease.  Later in the disease, they will have difficulty moving their eyes upward or downward completely causing them to spill their food when they eat or having difficulty descending stairs.  They will tend to decrease the amount of blinking which will lead to dry eyes.  The speech will become slurred making it difficult to understand what they are saying.  Much later there will be problems with their thinking resulting in a change in their personality as they become more forgetful, possibly cantankerous and indifferent.

This disease is a result of neuronal loss throughout the midbrain of the brainstem.  Later in the disease there will be significant neuronal loss within the parietal lobes and the regions of the frontal lobes responsible for initiating movements of the limbs.  The neurons demonstrate an accumulation of tau within the cell body prior to dying.  There is no known treatment to stop the progression of the disease.  There are medications to help ameliorate some of the symptoms.  The median interval from onset of the initial symptom to confinement in a wheelchair is 8 years.   

The results of a recent study reported in the most recent journal of JAMA shows that 2000 units of Vitamin E can have beneficial effects at retarding the progression of Alzheimer’s disease.  In an older study, 2000 units of Vitamin E was shown to be equally as effective as taking a prescribed medication (10 mg of selegiline) daily at improving activities of daily living (dressing, eating, walking) and delaying placement into a nursing home.  Interestingly, in this study if the patient combined taking both vitamin E and selegiline together the beneficial effect of either substance was lost.  There are additional studies which have shown slowed cognitive decline in the elderly without dementia if they take high doses of Vitamin E.  As mentioned at the start of this paragraph, a new study was performed which enrolled 613 patients with mild to moderate dementia (mean age 79; 97% men).  The patients were broken into four groups: 2,000 units Vitamin E, 20 mg memantine, Vit E + memantine, and placebo.   They were followed for 4 years.  The group taking Vitamin E alone showed a reduction in disease production of 19% per year.  It even did better than the memantine alone (although this was not statistically significant).  Interestingly, similar to the study that used selegiline, when Vitamin E was combined with memantine then the beneficial effect was lost.

Talk to your doctor about the benefits and risks of taking high doses of Vitamin E.  One major concern is that Vitamin E can thin your blood and for patients already on anticoagulants it may not be wise to add a high dose of Vitamin E as well. 

Vitamin E is a fat-soluble vitamin that is found in fruit, vegetables and whole grains.  It is best absorbed when taken with food.  It typically is dispensed in 400 IU’s capsules. 

This is a 34 y/o woman who over the past 6 months developed a clumsy right hand, loss of vision in her right peripheral field and slowing of her thinking.  During the past few weeks there had been some episodes of bladder urgency with some incontinence.  On exam, the right pupil was very slow to react to bright light from a flashlight.  She did not see as well in her right visual field.  The right side of her face was weak.  The right arm did not feel a pin as well as the left arm.   

This study demonstrated a large saccular aneurysm off the proximal intracranial left ICA.  This aneurysm is compressing the top of the left brainstem.  This part of the brainstem carries the impulses to and from the right side of the body (face, arm and leg). 

MRI brain with contrast

DIAGNOSIS: Left ICA Aneurysm 

The Affordable Coverage Act is rolling out but there are a few BUMPS in the road.   Here are some highlights …


1) There will be Universal Health Coverage.

REALITY

- Historically, when this has been attempted at the state level, it has failed.  Currently, there are 48 million in the US who are uninsured.  It is estimated that by 2019 the number will only drop to 31 million.

This is a 66 y/o man who experiences unusual episodes of weakness of the right leg – particularly when climbing hills.  It will become so weak that he will collapse.  Sometimes the weakness is accompanied by burning pain.   The examination is normal.  A MRI of the lumbosacral spine was normal.

I performed an EMG study which showed clear signs of a right L5 radiculopathy.  I reviewed the prior MRI scan of the lumbar spine and in reality there were signs for a possible dural AV fistula.  I sent him for an iodine angiogram study.

The angiogram shows a dural arterio-vascular malformation (AVM) supplied by an artery at the L3 level. 

DIAGNOSIS: Spinal Dural AVM

Clinical Exam (07/10): no longer symptomatic 

This is a 68 y/o man with a 4 month history of tingling of the left cheek with superimposed stabbing pain.  On exam, there is diminished pin sensation over the left cheek.  An initial MRI of his head was read as normal.

First, I performed a blink reflex in my office.  This is an electrical test in which I stimulate the trigeminal nerve and force an individual to blink.  I record how long it takes for a person to blink.  In this situation, there was a clear prolongation for blinking for the left lower eyelid relative to the right lower eyelid.  I then sent him for a detailed high-resolution MRI of the left eye.

This MRI scan shows a subtle lesion just under the left eyeball.  A biopsy of this tumor showed lymphoma.

MRI Brain

He was referred to an oncologist who treated him with systemic chemotherapy and he has been in remission for the past 6 years.  All of his facial symptoms resolved completely with treatment of the underlying lymphoma. 

DIAGNOSIS: Lymphoma 

What is Happening in the Brain of a Person with Alzheimer’s Disease?

Theory

A) General Injury

- All brains undergo neuroplasticity in response to damage.  Neurons are one of the few cells in the body that do not replicate.  When they are exposed to damaging ions (e.g. free radicals) following smoking a cigarette they repair themselves (use an intracellular repair protein called ‘tau’) and can branch to contact neighboring neurons. However, at some point in life this neuroplasticity will be overcome and these neurons that have branched together coalesce into a ‘neurofibrillary tangle’.