This time, Peter, a spry 55 year old man, has let his disdain for dentistry impair his better judgment. A known cavity had started irritating Peter 12 months ago. Initially, he ignored it but then as the gnawing pain worsened, he palliated himself with his collection of single malts, taken neat of course. However, the pain began to interfere with his ability to enjoy BBQ pork sandwiches and this he would not have. Off to the dentist.

There he learned that his tooth was not salvageable. It required extraction. “Fine,” barked Peter, who then uneventfully underwent a controlled tooth extraction by the skilled hands of his dentist.

Three weeks later, Peter had resumed life in his dual roles of both an obsequious accountant and a committed but generally annoying husband. At night one evening though, he began to have a fever. And over the next few days, this fever combined with an insidious malaise and dizziness. Quickly though, these symptoms began to pale in comparison to the headache he developed. The headache was constant and unremitting, unresponsive to every over the counter medicine he could purchase. When he came home from work that Friday though, his headache was bringing him to tears. He was sweating. He decided to take a seat on his prized leather couch and wait it out.

A few hours later, his wife returned home and found him with a vacuous stare on the couch, drenched with sweat. Further, she noticed that the left side of his face was droopy. When she asked what was wrong, he muttered some unintelligible syllables. When she asked him to get up, he made some clumsy gestures but did not move his left arm or leg.

At the hospital, Peter was much unchanged. He had a temperature of 40 degrees and a blood pressure of 90/50. An odor of bodily failure filled the room. Simply put, he looked terrible.

What’s going on? What to do?

Check back next week for the explanation.

Photo by PinkStock Photos

–

To summarize, Peter is a 55 yr old man with a history of having had a tooth extraction who now comes to the hospital with an acute neurologic illness consisting of fever, left sided weakness, and near coma.

The emergency room physicians astutely recognize Peter’s problem as neurologic in nature and thus organize a stat CT scan of the brain. Because of Peter’s symptoms of left sided weakness, we expect to find a lesion of some sort on the right side of his brain (Remember, the right side of the brain controls the left side of the body and vice versa).

Figure 1 – Note the arrow pointing to the circular lesion in right side of the brain (Sides are reversed on CT scans).

As pictured above, our suspicious indeed prove correct. Peter does have a lesion on the right side of his brain in an area that would certainly cause left sided weakness (For you hobby anatomists, the lesion is located medial to the genu of the internal capsule, abutting both the caudate nucleus anteriorly and thalamus posteriorly). Needless to say, the abnormality is in a bad spot as its bang next to the fibers that send control signals from the right brain to the left body.

At this point, we know that Peter appears ashen and has a circular lesion pushing into his brain. Because of his co-morbid symptoms of fever, sweats, and few day history of feeling unwell, we can justifiably surmise that Peter has developed a blood infection and an abscess in the brain, likely originating from the tooth extraction he had undergone weeks prior.

Blood infections(bacteremia and/or sepsis) after dental work are thankfully rare phenomena. Interestingly, after any tooth extraction, periodontal surgery, and even tooth brushing, we all have a bit of bacteria enter our blood stream. However, our immune systems are superbly effective at ridding us of these pathogens, usually within 30 minutes of the event. In fact, if you think of the number of times you brush your teeth in a lifetime, the risk of a significant blood infection related to your teeth is vanishingly small. In certain populations of patients, such as those with artificial heart valves, however, the risk is slightly higher. These bacteria can latch onto these prosthetic devices, grow and cause a condition known as infective endocarditis, which if left untreated can lead to a life threatening infection and heart failure. In Peter’s case, he was just extremely, unbelievably unlucky.

Peter is in serious danger. In addition to the infection in his body, he has an organized collection of bacteria(abscess) pushing into his brain. Because this abscess is not supposed to be in the brain, the pressure in the brain increases. When the pressure in the brain increases, neurologic symptoms ensure, which include weakness and eventually coma and/or death if the pressure gets too high.

As such, Peter needs emergent surgery. Because the abscess is located deep in the brain, traditional brain surgery to open the brain at the surface and remove the abscess is not an option, as gaining access to the abscess would endanger important neural structures. Instead, Peter needs a more minimally invasive approach wherein the abscess can be drained with only minimal damage to surrounding neural structures.

As such, Peter underwent a so-called stereotactic drainage of his abscess. In this procedure, a special CT scan is performed which is then loaded into a computer in the operating room. Then using fixed external landmarks on Peter’s head, the location of Peter’s head in real space is married to the images of Peter’s head in virtual space. In doing this, a special wand can be used during surgery to point to exactly where we are in Peter’s brain as the surgery is underway. More importantly, though, it will allow us to plan our placement of a catheter into the abscess and drain it while avoiding other critical structures.

Peter’s operation went smoothly. With the aid of surgery and antibiotics, Peter recovered over the next few weeks. However, he was left permanently weak on the left side of his body due to the damage the abscess wrought on his brain. For us doctors, it was satisfying to know his life was saved but sobering to realize we could not undo all the havoc caused by the infection. For Peter and his family, they were grateful for his survival but appropriately contemplative. Why did this rare event happen to Peter specifically? Why couldn’t modern medicine not only cure his abscess but also fix the weakness in his arm and leg? Why did Peter wait so long to have his tooth examined? Would it have mattered if he had?

The truth is, we just don’t know.

The post (Updated with Answer) The Medical Challenge: Can You Explain These Symptoms? appeared first on The Faster Times.

This year Regina has decided to exercise in the evenings after her shop closes. One day, she leaves for the gym and begins to have a slight headache. She shakes it off and decides to go running. As she begins to run, her headache suddenly explodes in intensity. She gets off the treadmill and immediately feels faint. She falls to the floor and suddenly cannot understand what is happening. She becomes nauseous and then violently vomits. Regina is now in a coma.

–

In the emergency room, Regina is unresponsive. She does not rouse when stimulated. When a painful stimulus is applied, she abnormally extends both her arms in response as opposed to grimacing or swatting away the painful stimulus. When a light is shined into her eyes, her pupils react quite sluggishly. Her breathing is irregular and alternates between shallow and deep. The doctors believe her breathing to be so abnormal that they decide to place a breathing tube in order to secure her airway. Now she is on a ventilator.

What happened? What should the doctors do next?

–

The Emergency room doctors have to act fast. Clearly, Regina is in critical condition. The most striking feature of Regina’s condition is her inability to interact with the outside world. Her responses to noxious stimuli are extremely abnormal. Because of these clinical features, the ER physicians order a CT (Computed Tomography) scan to evaluate her brain as her clinical condition strongly suggests a neurologic cause.

In the CT scan shown, the cause of Regina’s condition comes immediately into view. She has suffered a spontaneous subarachnoid hemorrhage from a ruptured aneurysm in the brain.



Subarachnoid hemorrhage is a serious condition which can result from trauma, blood vessel malformations in the brain, and aneurysm ruptures as in Regina’s case. An aneurysm is a weakening in the wall of an artery that generally results in an out-pouching of the arterial wall. Over time, the wall of the aneurysm that keeps blood from spilling into the brain weakens and may eventually rupture. Upon rupture, patients can have a variable set of symptoms from a mild headache to coma to even death. In fact, the pre-hospital lethality of subarachnoid hemorrhage may be as high as 20-40%. If the aneurysm is not treated initially, the risk of re-bleeding from the aneurysm is 20% within the first two weeks after the initial hemorrhage.

Subarachnoid Hemorrhage is a certainly a major neurologic health problem in the United States. Its incidence (medical term used to define the frequency of a disease in a given population) approximates 10 per 100,000 though this incidence varies greatly by the specific population studied. For example, the Japanese have the highest frequency of subarachnoid hemorrhage in the world. The annual cost of this hemorrhage approach nearly 2 billion USD per year. Risk factors for subarachnoid hemorrhage are multiple. Females, smokers, and cocaine users have a relatively increased risk of suffering a hemorrhage compared to the rest of the population.

Subarachnoid hemorrhage requires emergent treatment. In Regina’s case, the hemorrhage has caused the water tanks in her brain(termed ventricles) to swell due to the clogging effect the blood has on the brain’s ability to reabsorb its natural fluid. As the brain does not know to stop producing its own natural fluid, the ventricles increase in size and the pressure in the brain dramatically rises. This increase in pressure can case a variety of symptoms including headaches, blurry vision, confusion, lethargy, coma and even death if left untreated. For Regina, she requires an emergent catheter placement (ventriculostomy) into these water tanks(ventricles) performed by a neurosurgeon. This requires a small incision in the scalp, a small hole drilled in the skull, and finally the catheter inserted into the ventricles.

Once her catheter has been placed, Regina has two treatment options. The first is surgery wherein a neurosurgeon finds the aneurysm and places a clip across the neck of the defect. The effect of this clip is to seal off the aneurysm from the normal artery and thus prevent any future re-bleeding or recurrence of the aneurysm. A second option termed endovascular coiling involves placing a catheter into an artery of the leg and then feeding it through the body’s natural blood vessel pathways into the brain using x-rays as a navigation system. Once the catheter is sitting next to the aneurysm, metal coils are placed into the pouch of the aneurysm. These coils then cause the blood to form a scab around the coils which effectively seals off the aneurysm from the normal artery. The best treatment option depends on the size, shape, location, and particularities of the individual patient.

Once the aneurysm is treated, however, Regina has a long road ahead of her. She will remain in the intensive care unit for at least two weeks at which time her entire body will need intensive optimization by the ICU doctors, Neurosurgeons, and nursing staff to prevent strokes in the brain and other end organ damage. Her recovery from this hemorrhage is uncertain. Perhaps one third of patients like Regina who survive can go on to make a great recovery, another one third will recover with mild to moderate disability, and the remainder will survive with profound disability requiring constant nursing care.

Still, despite these statistics, modern day management and research regarding subarachnoid hemorrhage is helping to improve patient outcomes. More importantly, though, careful vigilance regarding your general health and the availability of emergency medical care are the most important pieces of this complex puzzle.

Links:

www.Stroke.org – National Stroke Association Website

www.bafound.org – Brain Aneurysm Foundation Website

The post (Updated with Answer) The Medical Challenge: The Headache of a Lifetime – What Should the Doctor Do? appeared first on The Faster Times.

George is a 46 year old painter from Scranton, Pennsylvania. He is a jovial fellow well-liked by his peers. When not painting, he and his friends revel in the delicacies specific to Northeastern Pennsylvania such as pierogies and Old Forge White Pizza. In addition, George is a significant smoker. Since the age of 15, he has smoked approximately one pack per day.

Over the last few years, George began to develop bizarre sensations in his hands and feet. He thought nothing of these, though, and ascribed these problems to old age. More recently, however, he began to notice cuts and bruises on his hands and arms at the end of his workday. Such injuries were not out of the ordinary in his line of work but strangely George did not notice these injuries when they occurred. Moreover, they did not particularly hurt.

One day, George awakens from his characteristically intense slumbering and realizes his vision is blurry. Specifically, he is seeing double and cannot seem to fully open his right eye. He decides on this day that it is finally time to seek the attention of a physician.

The last time George saw a physician, he was 15 years old. As such, he visits the local emergency room. The nurse checks his vitals.

Height: 5’10

Weight: 225

Body mass index: 32.3

Heart Rate: 85

Blood Pressure: 155/95

His Physician, Dr. Jankowski, reviews these vital statistics and proceeds to examine George after first discussing his symptoms and past medical history. On exam, he notices that George’s right eye is deviated and fixed towards the right side of his eye socket. When he asks George to move his right eye, he cannot though his left eye moves properly. In addition, George reports seeing double except when he looks toward the right. When Dr. Jankowski shines a light in George’s eyes, both pupils appropriately become smaller.

The top figure indicates the appearance of George’s eyes when he enters the Emergency room. In the bottom figure, the physician elevates his right eyelid and asks George to look towards the left which he cannot do. (Photos courtesy of Canadian Family Physician-www.cfpc.ca)

Dr. Jankowski proceeds to check George’s heart, lungs, and abdomen and finds no problems. Further, George appears to have good strength in his arms and legs. However, when Dr. Jankowski pokes George with a safety pin, George is barely able to perceive the pin particularly in his hands, forearms, shins, and feet. Finally, when George removes his socks, Dr. Jankowski notices a chronic appearing wound on the bottom of his left big toe. George reports being unaware of the wound.

What’s going on? What should we do?

Check back on Monday for the answer.

–

Dr. Jankowski immediately understands George’s affliction. First, upon looking at George’s vital statistics, Dr. Jankowski notices that George is both overweight and hypertensive(high blood pressure). Then he thinks about George’s story. George has had a few year history of strange sensations in his extremities. Furthermore, He has a chronic, non-healing wound on his foot. Finally, he has an acute problem with the movement of his right eye. These signs and symptoms point to problems with George’s nerves and given George’s age, lifestyle, weight, and blood pressure, Dr. Jankowski suspects George to have a severe diabetic neuropathy from complications of type II diabetes.

Diabetes Mellitus is a group of disorders characterized by high blood sugar that can ultimately lead to complications with the eyes, nerves, arteries, and kidneys of the human body(this is only a partial list). George’s specific diagnosis is that of type II Diabetes which is thought to affect nearly 25 million Americans. Moreover, at least $130 billion dollars in direct and indirect diabetes care costs are spent in the US every year. Sadly, the disease is the leading cause of blindness in adults, end stage renal disease(requiring either dialysis or kidney transplant), and non-trauma related limb amputations. The causes of this disease are heterogeneous and complex but put simply, genetics that predispose one to high blood sugar are mixed with a lifestyle that impedes the body’s ability to maintain proper sugar balance. Lifestyle factors of relevance in George’s case include his excessive caloric intake, poor exercise routine, obesity, and cigarette use.

Because George has had undiagnosed diabetes for likely quite some time, he has had damage to the nerves and arteries(and potentially other organs) in his body. Classic for diabetes, the nerves in the hands, forearms, feet and shins become diseased and essentially dysfunctional. This explains George’s strange sensations in his hands and feet, and his more recent symptoms of numbness in these areas. Moreover, because George cannot feel his feet adequately, he is unaware of the repetitive stress he suffers to his feet from everyday living. As a result, he has developed a chronic non-healing wound known as a diabetic foot ulcer. The wound has a difficult time healing because damage to his blood vessels from diabetes has severely limited blood flow to the injured area of his foot. Without blood flow, the body cannot heal the wound leading to the foot ulcer and potentially life threatening infections.

Similarly, George’s problematic right eye results from damage to the nerves controlling eye movements. There are three nerves that control movements of the eyes and the long standing nature of George’s diabetes has impaired the so-called Cranial Nerve III without which normal eye movements are impossible. In addition, that same nerves helps control the muscles that elevate the eyelid which if compromised results in a droopy eye like George’s.

George is therefore in trouble. He needs an aggressive change in his lifestyle consisting of exercise and diet control. He is significantly overweight as indicated by his Body Mass Index(to compute yours, go to http://www.nhlbisupport.com/bmi/ ). He needs to quit smoking which in addition to its attendant cancer risk also compounds the damage to blood vessels already caused by his diabetes. At this stage, he needs medicines to not only treat his high blood sugar but also control his high blood pressure. Finally, he needs to get a primary care physician to help him manage his diabetes as this will be a problem that requires lifetime care.

The good news is that George’s eye problems will likely resolve in 6-8 weeks. However, George’s numb, achy hands and feet may not get much better though pain in these areas can be well managed with certain medications. Improvement, though, is possible and George getting his blood sugar under rapid control optimizes his chances. With regards to his foot ulcer, nearly 15% of diabetics develop a foot ulcer and of these, perhaps 10-20 % may eventually require an amputation to prevent gangrenous complications. Treatments, thankfully, do exist for foot ulcers and include aggressive management of the diabetes, local wound care(using a variety of dressings and gels/creams), and antibiotics if necessary. Sometimes, surgery is required to debride the wound for optimization of wound healing.

The message here is that type II diabetes and its complications are largely problems that can be minimized, even avoided through a combination of lifestyle modifications and medical vigilance. In fact, cases of type II diabetes caught early can in some instances be cured by exercise and diet alone. Unfortunately, the complications George has suffered from his diabetes represent those of a more advanced stage and only a partial list of those possible. Finally, at this advanced stage of his diabetes, reversal of all his symptoms to his pre-diabetic state is generally not achievable. George must now learn to live with and manage his diabetes for the rest of his life.

As a society, we can do much to prevent diabetes through education and healthy lifestyle choices. For more information on educational materials, activism, dietary hints, and opportunities to support diabetes research, please visit the American Diabetes Association at www.diabetes.org

The post (Updated with Answer) The Medical Challenge: George’s Unnerving Surprise appeared first on The Faster Times.

The “Medical Challenge” is a new column by Dr. Rohan Ramakrishna. The cases described are based on real patients. Please leave your thoughts on this case in the comments, and check the comments again next week to learn the real explanation.

Julie laughs a lot. At six years old, she has always been regarded as the most cheerful among her two other siblings. Recently, however, Julie’s mother Sarah has noticed that her daughter seems to laugh for no reason approximately three-to-four times a day. These episodes of laughter last only seconds and follow seemingly banal periods devoid of humor. When asked about what makes her laugh, Julie reports not remembering.

A few months go by and Sarah notices that these laughing spells not only persist but seem to increase in frequency. Then Julie’s teacher calls a parent-teacher conference to discuss Julie’s increasingly aggressive behavior towards her peers and teachers at school. Julie subsequently received a firm scolding from her parents and was told to “behave.” Julie, of course, denied culpability and insisted she had done nothing wrong. She was subsequently grounded.

Three months later, Julie’s laughing fits and aggressive behavior have not subsided. Sarah is confused and worried. She decides to visit her pediatrician.

Sarah sits anxiously in her pediatrician’s office as she watches Dr. Ray examine Julie. Julie answers all his questions appropriately but has two laughing fits during her physical exam. Dr. Ray recognizes that these episodes are out of place. Dr. Ray also notices that Julie’s hands seem bigger than would be appropriate for her age. He orders an X-ray of her hands, which reveals a bone age of 10, well above her actual age of 6. Dr. Ray now believes he has an important clue.

If you were Julie’s pediatrician, what would you do next? What might explain her strange symptoms?

Answer

An astute pediatrician, Dr. Ray suspects precocious puberty and diagnoses this entity through his physical exam and bone x ray showing advanced bone growth. In addition, Dr. Ray recognizes Julie’s laughing fits as inappropriate and suspects these episodes require further evaluation. As a next step, he orders an MRI(Magnetic Resonance Imaging) to evaluate the brain as most causes of precocious puberty originate from central sources(ie brain) rather than peripheral sources(ie glands).

Julie’s MRI is nothing short of surprising.

In the nearby graphic(courtesy Barrow Neurological Institute), the arrow indicates a lesion in the hypothalamus of the brain. The hypothalamus is one of the master regulators in the body. Its functions are diverse and include regulation of hormonal, behavioral, and homeostatic functions. In short, it is like a car computer that makes sure all the different parts are efficiently coordinated and working appropriately. In Julie’s case, her MRI demonstrates a lesion known as a hypothalamic hamartoma.

Hypothalamic hamartomas are benign tumors that are classically known to cause gelastic seizures. Gelastic(from the greek “gelos” – to laugh) seizures are seizures characterized by mechanical bursts of laughing without antecedent environmental cues or an internal feeling of humor. These hamartomas are seizure inducing and the seizures they induce are often characterized by these aforementioned laughing fits. In addition, these seizures can be associated with disorders in personality manifesting as increasing aggression among other unpleasant personality traits. Moreover, either as a result of the tumor or the seizures themselves(likely a combination of both), people afflicted with this condition can have worsening cognitive performance and memory decline. Finally, these seizures tend to evolve with time sometimes turning into generalized seizures characterized by the unconscious stiff shaking with which most people are familiar.

Because of the tumor’s location in the hypothalamus, it can disrupt normalendocrine function. In Julie’s case, it caused an abnormal release in hormones related to sexual maturation leading to her increased growth and precocious puberty. Sometimes this can be effectively treated with medicines that effectively discourage release of these hormones.

In terms of treatment, Julie has essentially two options. The first is to treat with anti- seizure medicines to determine whether or not her symptoms improve. The second involves neurosurgery wherein the tumor is carefully removed. In addition to the usual risks of any medical procedure, the risks of surgery here involve permanent memory deficits and potentially permanent disruption of endocrine function requiring lifetime hormone replacement therapy. In experienced hands, however, surgery is a safe and effective therapy that can drastically improve the quality of life in patients with these tumors. As with every patient, though, a careful discussion of the risks and benefits of various treatment options needs to be properly undertaken prior to any treatment decisions.

Finally, it is important to note that hypothalamic hamartomas are extremely rare entities with guestimates placing their prevalence at 1 in a million. So next time you laugh, there’s no need to worry.

Photo by peyri

The post (Updated with Answer) The Medical Challenge: The Case of the Girl Who Laughed Too Much appeared first on The Faster Times.