When and how to seek a second opinion-a patient’s perspective

When and how to seek a second opinion-a patient’s perspective

 

NK Sethi ¹, PK Sethi ²

 

¹ Department of Neurology, Comprehensive Epilepsy Center, NYP-Weill Cornell Medical Center, New York, NY (U.S.A.)

² Department of Neurology, Sir Ganga Ram Hospital, New Delhi (India)

 

 

 

 

 

 

 

Address for Correspondence:

NK Sethi, MD

Department of Neurology

Comprehensive Epilepsy Center

NYP-Weill Cornell Medical Center

525 East, 68^th Street

New York, NY 10021 (U.S.A.)

Email: sethinitinmd@hotmail.com

 

 

There are times when a second opinion is not only appropriate, its necessary. This is true both from the patient’s as well as the doctor’s perspective. Since the patient technically has more to lose, it is imperative that patient’s know when and how to seek a second opinion. This is more significant in clinical neurology especially when one is handed down a diagnosis of a neurodegenerative condition like young onset Parkinson’s or Huntington’s disease. Diagnosis of a disease like amyotrophic lateral sclerosis (ALS) is essentially like signing off on a death sentence. Patients and caregivers are distraught and may not know what to do. Some may trust their doctor and agree to his or her management plan. But what if he is wrong? Maybe there is something out there that may help me. Maybe my doctor does not know about it. Even if the diagnosis is correct some may not be comfortable with the line of care. It is at times like these that the question of seeking a second opinion crops up.

Is my doctor right? is frequently he first question that comes up in the minds of many patients when handed down a diagnosis of a chronic or life threatening illness. Could he have made an error? Patients and caregivers may approach this in a couple of different ways. Some will inherently trust their physician skill entrusting themselves to his care. Others may seek to reassure themselves of the certainly of the diagnosis in one of many ways either by asking more questions of their physician or seeking information on the Internet. A fraction may decide to seek a second opinion. A question, which arises in the minds of some patients and caregivers, is would my doctor mind if I request a second opinion. Would he take it personally? Most doctors do not get upset if their patient requests a second opinion but some do. Many doctors may actually encourage their patients to get a second opinion especially if they have a rare condition or an atypical presentation. In these times of increased medical litigation, one form of defensive medicine practiced by doctors is to get a second opinion.

Now the question arises whether you want your doctor to suggest a specialist or do you want to do the spadework yourself. Having your doctor refer you to a specialist for a second opinion has many advantages. It may cut down on your time and effort and more importantly ensure that you are seen by someone who truly is a specialist in the malady that plagues you. Your own doctor may be willing to pick up the phone and call the specialist to apprise him of your case history. Relevant investigations can be quickly faxed to the specialist office. This ensures you are seen in a timely manner. Importantly the specialist has all pertinent records including results of tests down at his disposal at the time of your visit. Remember if you go to see a specialist without lab results, his opinion is at the most limited.

One must ask oneself what do I seek from the second opinion? Is it confirmation of my diagnosis? My diagnosis is confirmed but I want to know what treatment options are available or I just want a better explanation for my disease. Stick to what you seek from your second opinion and do not get side tracked.

Go prepared at the time of your second opinion visit. As your time with the specialist is going to be limited so make the best of it. Have your case history summarized. A good way is to have it typed out in a chronological order. When did the problem start, how did it progress and the treatment options that have been pursued. This shall save precious time and ensure that the specialist has all relevant data at his disposal prior to giving a second opinion.

Are there any cons to seeking a second opinion? While there are no real cons to seeking a second opinion, certain issues should be borne in mind. Remember there is no guarantee that a second opinion is right. The specialist may or may not voice the same diagnosis as your primary doctor. One can get side tracked and end up wasting precious time and money. Time and money that could have been used to begin treatment earlier. Do not get into the trap of doctor shopping, shopping till you get an opinion that you want to hear. Too many opinions have the potential for confusing you and leaving you undecided.

Seeking a second opinion is your prerogative as a patient but use it wisely.

ALS: dealing with terminal issues

It is June 29th Sunday as I pen this. I want to discuss about the issues which arise in the care of a terminally ill ALS patient. ALS as you know is a progressive neurodegenerative disorder characterized by the loss of anterior horn cell neurons in the spinal cord as well as in the brain stem (for more information read my earlier posts on ALS at http://braindiseases.info). As more and more anterior horn cells die, patients become progressively more incapaciated. They become weak losing first the ability to ambulate on their own, then wheel chair dependent and finally bed bound too weak to even feed themselves or take care of other activities of daily living. Throughout this their cognitive abilities remain relatively intact and they do not have memory issues. However as bulbar (brain stem) dysfunction occurs they are unable to speak and even unable to swallow or manage their own salivary secretions. Bladder and bowel function as well as eye movements are preserved till late in the disease course. No one quite knows why these functions are selectively preserved while the others are so affected. They also start having difficulty with breathing as they are unable to manage their salivary secretions and unable to cough if something enters their trachea.

As you can imagine the family as well as the patient have to deal with many issues. Thankfully there is much out there which can make life easier for these patients and their caregivers. Motorized wheel chairs are available which help in ambulation. Some of them are quite advanced and can even be operated by voice commands. Devices which serve as finger/ hand extensions are available and these can be of immense use to these patients if they want to pick something out like from the cupboard. Artificial voice devices are also available and hence patients are able to communicate their needs. Patients feel their independence is not lost and that is extremely important.

As bulbar functions deteriorate and secretion control becomes difficult it is important that something be done. Most of these patients need a tracheostomy finally but we can avoid it at least for a while with the aid of devices which produce an artifical cough. Mechanical insufflation-exsufflation devices (also known as In-Exsufflator, Cofflator, or cough machine) alternate positive and negative airway pressure to stimulate cough. These devices clear secretions by gradually applying a positive pressure to the airway and then rapidly shifting to a negative pressure thus stimulating a natural cough. Their use has been shown to decrease the occurence of respiratory infections in patients with ALS. Moreover they are non-invasive and easy to use.

Feeding becomes an issue and so is the maintainence of nurtitional status. While initially nasogastric tube feeding can be used, most of these patients finally need what we call PEG feeds. This is a tube placed by the surgeon into the stomach (the procedure is quite safe and simple actually). Once the PEG tube is in place, all the medications as well as feeds can be given via it.

I would advise patients and caregivers to discuss end of life issues at some point, even though these discussions are unpleasant and hard on everyone involved. Get in touch with the ALS association which shall be able to advise you further about devices out there which make life easier.

There is always hope and one must never lose it!!!

Nitin Sethi, MD

The human brain: the world’s foremost supercomputer

Read a fascinating post in the Wall Street Journal by By ROBERT LEE HOTZ titled ” Get out of your own way”. The author reports on studies and ongoing research into how our brain makes a “conscious” decision.

Let me take an example to explain the above. Suppose you are in a market place looking for apples. You are standing by the basket which is full of red apples. You look at the apples and are trying to pick just the “right” juicy ones. Then you make a choice and reach in and pick up the apple you wanted. Now you may think this is a conscious act, conscious decision making went into picking the apple. Research though shows that the human brain is far more superior and faster.

Even before you make the choice, the brain has already made its choice and knows which apple it wants from the lot. And that is the apple you finally pick up.

Functional MRI scans show that parts of brain are activated 10 seconds before the decision reaches the consciousness. This research sheds new light into the functioning of the human brain and the speed of its computation power. When the human brain is presented with choices, it is able to analyze countless permuations and combinations. How does our brain do this? How could the human brain defeat the worlds most advanced supercomputer in a game of chess. Surely the supercomputer would have been better with numbers and analyzing various possible chest moves. Like I said before, we still do not know how the brain actually works. But one thing which differentiates us from a machine is we think but we can think with emotions too. I can “cheat” in a game of poker or chess to throw you off. A computer once you understand how it works  or “thinks” you know it shall not work or “think” in any other way. A computer shall not cheat you.

A human brain on the other hand is never stationary. It has the unique ability to form new thinking processes. So now lets get back to our apple story. If the brain in few seconds has already made the choice it thinks is the right one (in this case the right apple) and that is the apple you finally pick up, then there may be some truth to the old saying of “going with your gut feeling”.

Overthinking a decision may not always be great. You go into a car dealership and are flip flopping over which car to buy. Thinking and rethinking your decision. Hmmm now you may say, it is a big buy and one must think through it well. True but the brain it seems has already decided and from what we know it has probably made the right choice as soon as you entered the dealership. If the first thing which crossed your mind was ” I want that red ford” well then probably that is what is right for you.

Now you may rethink and change your decision but it may not be the best one.  I find this concept fascinating and it just amazes me how the brain works. It is truly the world’s foremost supercomputer powered by God not intel!!!

Personal Regards,

Nitin Sethi, MD

Did you do your neurobics today?

Just wondering if you all did some neurobics today? Remember the brain needs to jog too everyday. So along with that body work out that brain. The nerve center needs some juice too!!!

Sweat those nerve cells everyone.

Developmental delay Vs developmental regression

Today I consulted on a 4-year-old boy who presented for evaluation of developmental delay.  As I tried to explain to the worried parents that their son had developmental delay due to a static encephalopathy, it dawned on me how hard it was for the parents to follow me. So here in this post I thought I would discuss the differences between developmental delay versus developmental regression. I shall also give an overview of the various causes of developmental delay and regression and how to go about investigating these children.

So lets keep this simple. What do we mean when we say developmental delay. Simple it means that the child is delayed in his or her developmental milestones. As we all know children acquire motor and language skills as they grow. These set of skills appear more or less at fixed times. For example a baby can sit by the age of about 8 months. Other motor developmental milestones include the age where the baby starts to support his head, crawl, roll over, stand, stand up without support, walk and so on. Pediatricians check for these milestones when you take the baby in for well baby visits. Similarly there are language milestones like when the child starts to speak  ma ma da da etc and when the child is able to form short sentences.

So it follows that developmental delay can be of a few types. A child may just have delay in the motor milestones, in others the motor milestones may have appeared at the right time but there is delay in language and other cognitive milestones. In still other there is delay in both motor and language skills (we refer to this as global devlopmental delay).

So what is developmental regression? Developmental regression is said to occur when a child who has been normal in his developmental and has met all the milestones starts to regress and starts losing the acquired milestones. Such a child may stop talking or stop walking and so forth.

As you can imagine the causes for developmental delay and those for developmental regression vary. Another couple of terms need clarification. We neurologists love to use terms called static encephalopathy and progressive encephalopathy in relation to developmental delay.

Static encephalopathy what does this mean? Let me explain with the aid of an example. Suppose you have a child who suffered some brain insult at or near the time of birth. Lets say he had hypoxia (lack of oxygen) to the brain. This child shall have developmental delayed but this shall remain static. The child is not going to progressively go downhill, infact as he ages if good intervention is carried out, it is more than likely that he would start to catch up wih his peers. He may never become completely ” normal” but he is not going to go downhill. His brain took a hit but now is trying to get back.

Progressive encephalopathy: let us assume another child. As compared to the first child, this child has a progressive neurological or systemic condition. Let us for example say he has a metabolic disease which leads to progressive brain damage. This child is going to go downhill as he ages. The brain is going to go “more bad” as time goes by.

Now that we have done with the medical jargon, let us try to make sense of some of the causes of developmental delay and developmental regression. There can be numerous causes of delay. The baby may have suffered some brain damage at or near the time of birth (during the antenatal period when the brain is developing or near the time of birth). Examples include conditions as diverse as mother abusing alcohol during the antenatal period (fetal alcohol syndrome), drugs used during the antenatal period may also harm the developing fetal brain. Hypoxia (lack of oxygen to the brain) is one of the common causes of cerebral palsy which presents as a static encephalopathy and developmental delay. This hypoxia may result from a difficult child birth or complications during labor and delivery. Inborn errors of metabolism, certain genetic disorders constitute other causes of developmental delay. Unrecognized seizures can also cause delay in development. Absence seizures is one such cause.

Developmental regression too has numerous causes.

How does one work up such children? The work up starts with a detailed history and physical examination. Your doctor shall go in depth into the birth history to find any cause. Family history is taken, where any drugs used during pregnancy? Then the child is examined to document is he delayed just in motor skills or is there global delay. After that the work becomes more complicated. Blood work to rule out inborn errors of metabolism, genetic and karotype testing, MRI scans of the brain and EEG may be ordered to zoom in to the diagnosis.

If the final cause is static encephalopathy, then there is nothing”active” to treat. What happened has happened. Now we need to concentrate on early intervention services to bring the child up to par. Physical therapy, language therapy, special education is what we need to stress on.

If the final cause is a progressive encephalopathy then of course depending upon the cause we need to treat. Is the cause treatable?

My advise to parents dealing with this difficult situation: find a good doctor, a pediatrician or pediatric neurologist who can help in getting to the bottom. Do not get disheartened, there are many interventions out there for your loved one. Seek treatment early rather than late as you want to halt a progressive encephalopathy early before too much irreversible brain damage occurs.

Nitin Sethi, MD

Stroke prevention: tackling the basics

Today I was having dinner when a friend asked me ” how can I reduce my risk of stroke?”. Briefly he explained to me that he had 2 heart attacks in the past. At that time he was found to have “slightly” high blood pressure and “bad” cholesterol. He said he was talking 2 medicines to lower his blood pressure and one to bring down his cholesterol. He could not provide me with further details and again I was suprised how little some of us know about our own medical condition. If I had asked him how his car was doing, I am sure he would have been able to furnish me a ton of detail. The mileage, the last oil change date, the date when the next oil change was due would have come rolling out.

Why do we neglect our own health? Is it just something we are not comfortable in talking about. Like a bad dream which one day when we wake up we shall not have to deal with any more. Some patients have inherent faith that God is watching out for them. This is particularly true in some cultures like for example in India, where some people feel that everything is God’s will. Hence they either detest taking medications or feel they do not need to take them. Some are lax with their follow up appointments. Other shall take the blood pressure and cholesterol lowering medications, only to self discontinue them when their blood pressure and cholesterol becomes ” normal”.

In any case, let me here tackle the basics in stroke prevention. Just straight talk, the kind you may not hear from many of your doctors.

1) IF YOU ARE A SMOKER THEN QUIT SMOKING. That is probably the single best thing you can do yourself to cut down your risk of a stroke as well as a heart attack. There is no excuse, like I have cut down from a pack a day to about 3 cigarettes a day. NO AMOUNT OF SMOKING IS HEALTHY. You should quit completely if you want to cut down your risks of a heart attack or a brain attack (stroke). Now I do understand it is difficult to quit but here I am talking straight and not beating around the bush.

2) Exercise: again a thing which you can do yourself to reduce your risks of a heart attack or a stroke. The exercise which is recommended is more the aerobics kind. The idea is to increase your heart rate and sustain it at this level for some time. Running or brisk walking all are beneficial. Now before you do embark on a exercise program, run it past your doctor to make sure that you are fit enough to exercise. You do not want to strain a weak heart and exercise ideally should be graded. YOU START SLOW AND BUILD UP AS YOUR BODY GETS USED TO IT.

3) Blood pressure: we have known for a while now that elevated blood pressure is one of the most important risk factors for strokes.  Recent studies have shown that the lower down you can get the blood pressure to, the more are the benefits. Earlier we used to advise our patients to aim for 130/80 mm Hg, now we say go for 120/70 mm Hg especially if you have other risk factors like stroke or previous history of coronary artery disease. There are many different medications that can be used to bring down the elevated blood pressure but some are recommended more because they have other added benefits apart from just lowering the blood pressure. Prominent among these is a group of medications called the ACE inhibitors. My advise to you shall be to get your blood pressure checked and than have your doctor decide which blood pressure medication shall be the best for you.

5) Bad cholesterol: bad cholesterol may run in your family that is it might be genetic in etiology. So not all bad cholesterol is your fault and due to the fact that your diet is bad.  In either case it is one of the major risk factors for strokes and heart attacks. When we measure the cholesterol in the blood ideally we should measure it in a fasting state (so the blood sample should be taken when you are fasting like in the morning before breakfast). We measure the total cholesterol level in the blood and then measure the differrnt fractions of cholesterol. Some as you know is what is called good cholesterol, this is called HDL or high density cholesterol. The other is bad cholesterol, the one which we want to be lower ideally. These include LDL or low density lipoprotein, triglycerides or TG and very low density lipoprotein (VLDL) among others. The goal is to raise the good cholesterol or HDL and lower the bad cholesterol like TG and LDL.

We aim for total cholesterol less than 200, LDL less than 100 (nowdays we are even more aggressive and may aim for it to be less than 70) and TG less than 200 too. These goals can be achieved by a combination of dietary modification, exercise and medications. Fish is rich in omega 3 fatty acids and raises your HDL or good cholesterol. So replace the red meat with more fish. There are a number of medications out there. The most commonly used class is called statins and they  help to raise your HDL and lower your LDL by a few points. However if your TGs are elevated, you may need a different class of drug.

My advise get your lipid profile done in a fasting state, then discuss the results with your doctor and have him help you decide whether you need just dietary modification or drugs too.

6) Control your blood sugar: if you are a diabetic this is very important because diabetes too is a risk factor for stroke. My advise get your blood sugar checked frequently both in the fasting and post prandial (after eating) state. The goal is fasting blood sugar less than 107 and post prandial less than 200 (in the case of post prandial it depends upon the time after eating when the sugar is measured). Your doctor may also check your Hemoglobin A1 C, the ideal goal for this is less than 7. Recent study in New England Journal of Medicine suggested that too aggressive lowering of hemoglobin A1C causes more harm than good (if you lower it less than 6) and may infact increase mortality. Hence again speak to your doctor and determine your goals.

7) Reduce your stress: mental stress is a killer and nothing is more important than your health. Do meditation and whatever else that works for you.

8) Drink alcohol in moderation: old saying too much of anything is bad and that includes the spirits!!!

 REMEMBER A STROKE PREVENTED IS A BRAIN SAVED!!!

Nitin Sethi, MD

Spinal cord lesions in MS

Multiple sclerosis is a demyelinating disease of the central nervous system (demyelinating because the disease is characterized by the loss of the myelin sheath around the axons of the nerve cells). As I have stated in my previous posts on MS (see http://braindiseases.info for all the previous posts on MS), the disease is characterized by plaques which are disseminated in space and time.

Most of these plaques (demyelinating lesions of MS) are seen in the brain but a few patients have what is loosely called spinal MS or rather MS in which the plaques are more commonly seen in the spinal cord (remember the spinal cord is a part of the central nervous system). These patients with spinal MS present with slightly different clinical signs and symptoms. They may present with what is called transverse myelitis (this is an involvement of the spinal cord usually at the cervical or thoracic level). Transverse myelitis can be devastating because all the descending motor fibers from the brain and the ascending sensory tracks are packed in the small diameter of the spinal cord. So any involvement of the spinal cord has the potential to affect all these tracks. Depending upon the level of cord involvement patients may have either weakness of just the legs (paraparesis or paraplegia) or all the four limbs may be involved (quadriparesis or quadriplegia). Usually the bladder and bowel are involved too and patients may have complaints of urinary incontinence. Sexual dysfunction is also commonly reported (erectile dysfunction in males, see my previous post on it).

As the involvement of the brain is less, these patients are relatively well preserved cognitively and may not have prominent cerebellar findings.

Spinal involvement in MS is treated in much the same way as other forms of MS. Your doctor may use a course of intravenous corticosteroids if you present to the hospital with acute transverse myelitis. Immuno modulating drugs like interferons may later be prescribed.

Personal Regards,

Nitin Sethi, MD

Erectile dysfunction-some neurological causes

I thought I would discuss some neurological causes of erectile dysfunction and decreased libido. There are many neurological diseases which are associated with erectile dysfunction and may also result in decreased libido. One of the most common is diabetic autonomic neuropathy. Diabetic patients especially those who have autonomic neuropathy (dysautonomia) frequently have erectile dyfunction. Supprisingly many do not volunteer this information, unless their doctor asks about it. They may complain of other symptoms of neuropathy like pain and numbess in their hands and feet but may not volunteer the history that they are having erectile dysfunction. Many patients do not realize that their erectile problems are a part and parcel of their uncontrolled diabetes.

Erectile problems and decrease libido is also frequently seen in patients who have multiple sclerosis. Fortunately this aspect of MS is now been given increasing recognition by doctors and a discussion is initiated with the patient at some stage of their treatment. Women with MS may have complaints of decreased vaginal lubrication, loss of vaginal muscle tone and diminished clitoral engorgement.
All this can lead to a decrease or loss of sex drive. Decreased or unpleasant genital sensations may lead to a diminished capacity for orgasm. Men with MS experience  erectile dysfunction and a decrease in or loss of ejaculatory force or frequency.

Erectile dysfunction and sexual difficulties are also a part and parcel of certain neurodegenerative conditions like multi system atrophy especially a syndrome called Shy Drager Syndrome in which autonomic failure is prominent. Parkinsons disease and patients with degenerative dementias may also have some of these problems. In these patients the cause is usually multifactorial.

Patients with epilepsy also frequently have sexual dysfunction. Again the causes are multifactorial but one important reversible cause is drug side-effects. Some anti-epileptic drugs and antidepressants frequently cause sexual dysfunction as a side-effect.

The good news is that sexual dysfunction is now more readily recognized as a part and parcel of certain neurological disorders. Neurologists are nowdays more adept in asking patients about it. It is important that patients volunteer information if they are experiencing sexual dysfunction as many of the causes are treatable. Drugs with sexual dysfunction as a side-effect can be stopped and replaced with other drugs. Also some of the symptoms can be ameriolated by using medications like sildenafil (Viagra).

Personal Regards,

Nitin Sethi, MD

Stroke rehabilitation

I thought in this post I shall discuss a little about stroke rehabilitation. A stroke can be devastating and causes significant morbidity and mortality. Stroke survivors are frequently left behind with neurological deficits. Common deficits include: motor (weakness on one side of the body usually arm and leg), speech deficits (patients may be left with a significant disorder of language. We call this aphasia and patients may have either difficully in comprehending speech or in the fluency of speech. Their speech may be slow and halting. At times they also have what we refer to as nominal aphasia also called anomia. These patients experience difficulty in naming things, like for example if you show them a pen, they would not be able to tell you that it is a pen. They shall know what it is used for but cannot get the name out). Patients may be left behind with residual numbness on one side of the body and depending upon the location of the stroke they may experience difficulty with seeing one half of their visual field (we call this a field cut). Some stroke patients are disabled by ataxia or unsteady gait, others may have disabling cerebellar symptoms.

Stroke rehabilitation plays a big role in the care of stroke patients. Contrary to popular held views, rehabilitation starts right at the onset of stroke and not once the acute stroke is over and the patient is been discharged. Now most of the big hospitals in the United States have acute stroke units. One of the biggest tenants of these acute stroke units is that rehabilitation is started soon after the patient presents to the hospital with an acute stroke. Studies have shown that the human brain has the maximum ability to heal and repair itself acutely (upto 6 months to a year) after a stroke. As time goes on, the brain loses this plasticity and the neurological deficits become more or less fixed. Thus  rehabilitation in the acute stroke setting plays a vital role. Usually patients are evaluated by physical medicine doctors (rehab doctors) while they are still in the hospital with the acute stroke. An assessment is made of their deficits and depending upon that we decide which rehabilitation services are needed. Patients may get physical and occupational therapy in the hospital. They may also be seen by a speech and language therapist so that speech therapy can begin.

Nowdays there are many different modalities of rehablitation therapy which have been proven to be of benefit to patients with stroke. One of the popular ones is constraint induced movement therapy. In this the good arm of the patient is immobilized so that the patient is forced to use the weak arm. Melodic intonation therapy has recently gained popularity too. This therapy uses a style of singing called melodic intonation to stimulate activity in the right hemisphere of the brain in order to assist in speech production in patients who have aphasia.

Stroke rehabilitation does not end once the patient is discharged from the hospital. Most of these patients benefit from ongoing rehab (as I stated earlier this mostly occurs in the first 6-12 months). Patients may either be discharged to a rehab unit or a sub acute rehabilitation facility where these therapies can be carried out in a less hospital like atmosphere.

It is Saturday afternoon New York time. I though pen this from across the seven seas (I am on vacation!!). Hope some of you find this useful.

Personal Regards,

Nitin Sethi, MD

Normal pressure hydrocephalus (NPH)

Normal pressure hydrocephalus (NPH) is another potentially reversible cause of “dementia” or rather memory problems in the elderly. NPH is characterized by the triad of gait disturbance, urinary incontinence and memory problems. The dementia in NPH is of the subcortical type meaning that it is mostly characterized by psychomotor retardation (patients are slow to respond), unlike cortical dementias like Alzheimers disease they do not have language deficits (aphasia), inability to do learned things (apraxia) or agnosia.

The etiology of NPH is still not fully elucidated but it is thought to involve some obstruction to the normal flow of the cerebrospinal fluid (CSF). This obstruction may be due to previous history of meningitis or CNS trauma. When you image these patients with either a CT scan or a MRI scan, one finds the ventricles are dilated (large enlarged ventricles) but there is not much corresponding cortical (brain0 atrophy. This is against what is found in cortical dementias like Alzheimers disease where apart from the ventricles been dilated and enlarged, the brain also is shrunken (atrophy).

So in which patients should the diagnosis of NPH be entertained? Mostly these are patients who are elderly and who have had a subacute onset of memory problems accompanied by difficulty in walking (patients who have NPH have a characteristic gait (they walk slowly and stiffly, we call their gait as magnetic gait). They may or may not have urinary incontinence (the entire triad may not be present in all the patients). Neurologists entertain the diagnosis of NPH if they see a patient with the above symptoms and if the imaging is characteristic.

To confirm the diagnosis though requires further testing. Normally what we do is a therapeutic as well as diagnostic spinal tap. What does that mean you may ask?

Well we bring the patient into the hospital and do a spinal tap. Before the tap is done the patient is examined to determine the memory deficits. A timed walking test is carried out ( we make the patient walk a fixed distance and measure the time taken to do so ). Then a good amount of spinal fluid is removed about 20-30 cc. The pressure of the spinal fluid is measured at that time and in a typical patient of NPH it should be normal (hence the name normal pressure hydrocephalus). Once the spinal fluid has been removed the patient is again tested. Has the memory improved. We make the patient walk the same distance as before. If now the walk is much faster and steadier, then we document a positive reponse to large volume CSF removal. Our diagnosis of NPH is now strengthened and there has also been a therapeutic response to the procedure ( remember I told you the test is both diagnostic and therapeutic).

Before I usually subject the patient to a surgical option for more definitive treatment, I usually like to repeat the above test at least two more times. If there is a consistent positive response to large volume CSF removal, then I feel confident in going ahead and asking the neurosurgeon to place a shunt.  What is a shunt? Well a shunt is a device which as the name suggests shunts the spinal fluid from the brain into the peritoneal cavitiy (the gut). It has a valve which can be set to open at a particular pressure. So whenever the CSF pressure rises above that pressure, the valve shall open and the extra spinal fluid shall be shunted from the brain into the gut.

Simple device but does have its own risk of complications. Shunts can get infected, they can get dislodged from the brain and start migrating, they may get obstructed and have to be replaced etc. Hence before I advise putting any sort of hardware into the brain, I try to be as sure as possible that my patient indeed does have NPH and not a cortical dementia like Alzheimers or Parkinsons disease (as these do not respond to shunt placement).

It is Friday the 20th, I am off home on my vacation but shall be keeping the blog active. Please do contact me if you have any questions or want me to discuss something particular.

Personal Regards,

Nitin Sethi, MD