The neurocognitive effects of Lyme disease: The standard of care is changing

Whittier Rehabilitation Hospital was host to an audience of school nurses and nurse case managers at a special presentation entitled “The Neurocognitive effects of Lyme Dsease”. The speaker was Dr. Michael Sefton, Director of Neuropsychology and Psychological Services at the hospital. Lyme disease was first recognized in 1975 when a cluster of pediatric cases of severe arthritis was identified and ultimately linked to the common occurrence of deer tick bites as a possible source of the infection. The disease is not new and similar variants of the infection have been seen in the upper mid-west and in Sweden. The standard of care has gradually changed especially in terms of definitive antibiotic therapy and the course of therapy.  Physicians who treat Lyme now prescribe a 12 week course of antibiotic often – oral doxycycline sometimes followed by 2 weeks of a broad spectrum antibiotic. With antibiotic treatment, physicians have greatly reduced the use of antibiotic medicine because of the growing problem of super bugs – less is more when it comes to prescribing antibiotic therapy.  
The Northeast has the highest incidence of Lyme disease in the country with 20,000 cases reported annually. According to the CDC, as many as 90 % of cases go unreported. Lyme disease can cause significant senility to those bitten by infected the Blacklegged deer tick – the most commonly found member of the Arachnid  family in the region. If you do the math that suggests the true incidence of Lyme may be as high as 150,000 to 200,000 cases annually. Arachnids share phylogenetic ties with spiders, mites, and scorpions. Meantime, the population of Lyme bearing ticks is growing. Ticks are arachnids and are carnivores deriving their nutrition from the blood of neighboring hosts. The life span of the Blacklegged tick is about 2 years and they feed 3-4 times during their lifetime. The disease is tough to treat and carefully administered treatment is needed to alleviate symptoms. 
“Lyme disease is caused by a spirochete—a corkscrew-shaped bacterium called Borrelia burgdorferi. Lyme is called “The Great Imitator,” because its symptoms mimic many other diseases. It can affect any organ of the body, including the brain and nervous system, muscles and joints, and the heart” according to the Lyme Disease Organization website.
We are referred Lyme disease cases a few times each summer here at Whittier although ticks are active year-round. The bite leaves a telltale bulls eye bite with a red ring that can grow as big as 12 inches. The bite can be prickly, hot, and painful but the worst part of getting bit by a tick is the risk of developing full blown Lyme disease. Physical symptoms are broad including flu-like symptoms, headaches, fatigue, muscle pain, joint pain, changes in sleep hygiene, and cranial nerve involvement. Over 60% of Lyme patients describe moderate cognitive impairment and very often poor mood regulation – sometimes Lyme rage – from the loss of mood stability in the brain. The claims of “Lyme brain” and “Lyme fog” are well documented and equally debilitating as the physical sequelae. 
The neurobiological underpinnings evolve from the Borrelia bacteria seeding across the blood brain barrier and other organ systems. I have a male patient who developed cardiac arrhythmia among his symptom profile and must now be treated for this. Once the bacteria enters the CNS the inflammation associated with the infection can be misidentified as dementia especially in the absence of the prototypic bulls eye coupled with the delay in getting treatment. The delay often stems from 2 factors: the first has to do with awareness.  Only 50% of people with tick bites have any memory of being bitten by a tick.  This makes it hard to prescribe the standard treatment for Lyme and second; diagnostic lab tests often come up falsely negative because of a lack of antibodies in serum to suggest that an infection is cooking. In some of my patients they have been seronegative for greater than 6 months before finally getting aggressive antibiotic treatment. A negative blood  test is nothing to celebrate when you are unable function because of debilitating fatigue, mental fogginess, slow processing, limited cognitive endurance, headaches, decreased working and short-term memory. 
Family pets often carry ticks and usually test positive for the Borrelia tick bacteria.
Photo by Julian Paolo Dayag on Pexels.com

I have had several patients suffer for over 3 years from the ravages of tick bites and have been told that some have died from the consequence of being bitten. There is now a “post-Lyme syndrome” for the 15 percent who have chronic symptoms last over 1 year after treatment. For them the decreased mental energy and foggy feeling of Lyme disease is compounded by months of frustration and delayed or incomplete treatment. Many require a period of disability where they are removed from their jobs and allowed to focus on the rehabilitation and finding themselves in all the unfocused fog, forgetting, and the new pace of life.

Lyme disease if a debilitating condition that starts with being bitten by Borrelia tick who infects its host sometimes as soon as 15 minutes after the bite. Symptoms can be severely debilitating including depression. The majority of cases have neurocognitve deficits that are co-occurring with the physical debility. Together Lyme disease is a formidable affliction that no person should experience. Here in the Northeast our primary care providers should become better trained in the diagnosis and treatment of Lyme and not leave the specialty care to the large medical centers often unavailable to the rural population.

Spatial planning and the clock drawing: Strange times in cognitive functioning

The clock drawing is one component of the Montreal Cognitive Assessment test published by Pearson Products here in the US. It is a useful method of screening for change in cognition among an older population. It is useful to assess cases of suspected dementia. The Clock of the week consists of an interesting array of hands pointing to each number.  I am unsure what triggered this 71-year old to craft the clock in this manner.  

When is see my cases at Whittier Rehabilitation Hospital in Westborough, MA I am usually struck by one person or another saying “Are you going to ask me to draw a clock?” The speech pathologists at WRH usually ask for a clock drawing in conjunction with the initial evaluation.

For whatever reason some of the patients who are assessed are able to remember the clock drawing and are surprised at its importance or utility in assessing cognitive functioning. Yet, if you look at some of the clocks I have published in the past 12 months they are certainly indicative of strange times in cognitive functioning. Most of the clocks I highlight illustrate a poignant depreciation in mental awareness and self-monitoring. People ask me “why does the patient have such trouble drawing a clock? Can’t they remember what a clock is? Why would they draw so many hands on the clock face?” The answer is the loss of functional capacity and its impact on everyday activities. If someone fails to correctly complete a clock drawing they will have difficulty at so many more tasks of independence.

Actually, the clock of the week today illustrates the decreased appreciation of error awareness and cognitive inflexibility. The clock itself looks fine but when the third step is required e.g. setting the hands, the patient lacks fundamental problem solving and error detection.

 

Traumatic brain injury that starts with a laugh, a shrug, and a trickle

It does not take a pounding head strike to cause a fatal TBI. It has been shown over and over that many cases of TBI are missed or even not transported after a little fall with or without a head strike. How is it possible that such a slight bump to the head can result in such an outcome? It starts with a trickle. That is to say even a slight blow to the head can cause a trickle of bleeding in the brain that may eventually be enough for a bad outcome. In the setting of slow bleeding in the brain the most common complaint is a head ache that grows increasingly worse, confusion, and eventually loss of consciousness. This is a true emergency and patients must be taken to the nearest trauma center within the “golden hour” after injury. Concussion is a blow to the head that results in changes in cerebral perfusion and metabolic efficiency. Concussions are nothing to scoff at as we have seen in scientific literature including a link to chronic traumatic encephalopathy (CTE). Brain injuries differ from case to case and as we have seen over and over all it takes is a fall to set the cascade into motion. In some cases no head strike at all may be sufficient to cause subdural or subarachnoid bleeding from the force of whiplash or the fall from a height. The wife of actor Liam Neeson died a few hours after falling on the bunny slope in Ontario, Canada on a ski holiday in 2009. She had sustained an injury resulting in a trickle of blood that slowly filled the intracranial space making it impossible for her brain to function. She was initially conscious and alert – even laughed at having fallen on such a slight hill. But no one could see what cascade of events had been precipitated by this benign event.
“Natasha Richardson sustained a head injury on March 16, 2009, while skiing with the couple’s two sons on a beginner slope on Quebec’s Mount Tremblant. At first the 45-year-old actress laughed off the mishap, but called an ambulance two hours later when her headache became much worse. She was rushed to a Montreal hospital, where doctors discovered an accumulation of blood between the brain and skull called an epidural hematoma. Ethan Sachs NY Daily News Interview with Neeson February, 2014
Image of lateral aspect of cortex and cerebellum
None of us knows just what happens in the hours following a concussion so we take each one seriously. Indeed, Natasha Richardson had a much more lethal injury than was first thought. It may have been survivable with early trauma care. This is especially important for older fall victims who may be receiving blood thinners and are at high risk for bleeding. In addition, some patients with cortical atrophy – the result of shrinking of the cortical mantle from age-related change add to the risk of bleeding in the brain.
Dr. Andrew Naidech, a stroke and neurocritical care specialist at Northwestern Memorial Hospital said it’s “not uncommon” for patients who suffer head trauma to begin to feel better, then, many hours later, quickly find themselves feeling much, much worse.    
In a 2016 Blog I described second  impact syndrome (SIS) that is a rare but generally catastrophic injury that results from a second blow to the head that immediately follows a concussive injury – often undiagnosed or an injury that was previously diagnosed but not fully healed. The second injury to an already damaged brain results in catastrophic swelling within the brain often incompatible with survival.  Scottish boxer Mike Trowell died of a severe brain injury occurring in a sanctioned boxing match in Glasgow, Scotland on September 29, 2016. Recovery from concussion can take 10-30 days. Symptoms include decreased mental endurance, fatigue, inattention, headache, sensitivity to sound or light, forgetfulness, irritability, sadness, feelings of being foggy, dizziness, vertigo, and a few others. I am involved with a Concussion Management Program at Whittier Rehabilitation Hospital in Massachusetts that provides an interdisciplinary approach to treatment. WRH also offers inpatient TBI Rehabilitation at its Westborough and Bradford locations in Massachusetts.

Lest we forget: The clock of the week

Here is the Clock of the week for April 25, 2019. It is a classic and speaks to the disorganization and confusion some patients experience with dementia. The task is a basic cognitive demonstration of understanding for verbal commands or directions. It requires the interpretation of that direction with the frontal initiation of a response to the requested task. In this case the clock was constructed left to right with numbers randomly assigned to each circle. All 12 numbers are depicted but the patient could not integrate them into a coherent whole nor set the clock to the appointed time per the protocol. Vote for the clock of the year in December!

Following directions in real time

In order to be functional you must be able to follow directions using cognitive skills. Put simply, our brain must be able to process information – especially verbal information such as: “Are you in pain?” or “Please stand up”. The problem lies in cases where patients have sustained an injury to their brain and must now take extra time to decode the commands and turn them around into action as described above. There are many conditions that interfere with psycholinguistic comprehension including aphasia, delirium, encephalopathy, and even poor hearing. The reader should never presuppose one of the former three conditions without first ruling out the effects of hearing loss.

What is meant by following directions in real time? If you have ever tried to tell a joke to someone with very poor hearing you must often repeat your words – sometimes over and over. The effects of this have negative impact on real time communications making it almost impossible to impart comedic spontaneity into any conversation for example. This results from the real time failure of verbal understanding or inattention to pragmatic communication, inefficient processing of the sequence of language, inability to concentrate on what is being said and its linguistic salience, or an inability to hear the acoustic sounds as they are being expressed. Real time comprehension refers to the processing of information without delay, as it happens.

A number of variables interfere with this such as the need for repetition, misunderstanding of direction, delay in processing the sequence of words and understanding, and limited capacity to hear and reprocess the commands using working memory. Problems in understanding directions occur from the breakdown on any of these features of speech, memory, and attention. A neuropsychological assessment will help tease out at what point the meaning of the communication is lost. Sometimes it comes down to hearing aides and actively engaging the patient in the task itself. If they are disinterested there will be limited compliance and resistance. I always suggest a bedside examination that allows for beginning rapport and establishing trust before attempting the formal examination.

Vote for clock of the year!

 

Dec15_18 Clock of weekScan
# 1  This was drawn by a female with decreased self-monitoring

Dio ClockScan
# 3  Clock drawing by 79-year old male with dementia.  # 2 clock is above.  Size is about 8 millimeters measurement is shown in centimeters millimeters

Clock of the Week 8-15-18Scan
# 5  64-year old right handed male with severe lung disease

Clock of Week Sept 25Scan

Square_PassingclockScan
# 6 Clock – A better rendition aside from square face

Lauren patientScan
# 7 Clock – Drawn without evidence of conceptual awareness

 

Clock of the week_Nov8
# 8 Clock shows signs of perseveration and deminished self-monitoring – the numbers go on and on

If you are interested in the clock drawing and casting a vote for clock of the year you may contact me at msefton@whittiehealth.com or by leaving a comment in the space provided at the bottom of the ballot.  I look forward to hearing from you.

Clock of the Year – Vote on your favorite

Dec15_18 Clock of weekScan
Clock drawing by intelligent 71-year old
Every year I publish a series of clock drawings. Some people really like seeing the range of clocks that people draw including the clock above – drawn by a 71-year old right handed woman with confusion. One could argue that she had much of the task drawn correctly until she tried to correctly set the hands as instructed. I like this clock because it illustrates the break down in problem solving of the three steps required for success. Ostensibly, this patients followed the directions as instructed. The numerals were correctly placed and finally she placed hands on the clock. Arguably, by drawing too many hands she may have been exhibiting a perseverative response, decreased self-monitoring and error detection, and a failure in understanding the problem in terms of the correct time – 11:10. In this case she asked “AM or PM” but did realize her error relative her specific drawing. She drew no circle into which the numerals were to have been placed.  The yearly ritual of finding the clock of the year has begun. What fun!
“For many living with a person who has dementia can be an unforgettable challenge that evokes guilt, resentment and despair.” Michael Sefton 2018
The clock above was drawn by a 71-year old female who is in the hospital because of an infection.  When this was drawn her infection had been cleared up.  She is a verbally gifted woman with a full education.  Her family has seen a change in her thinking skills in the past 12 months and now wonders what can be done? Her verbal intelligence is in the 90th percentile with an estimated VIQ of 120.  Verbal abilities often interfere with the assessment process and there comes a time in every assessment when the examiner must put a stop to the fun and frivolity of making acquaintance. 
This year I received a few more drawings from clinicians in the community including the clock of the week from December 10, 2018.  One of the clocks that was published this year was featured on the Australian Broadcasting Company television series “Ask the Doctor“. The clock is featured about 16 minutes into the broadcast. I have been given permission by the ABC to display the link to the broadcast. I will publish several clocks and I ask that readers vote on the most interesting clock.  We will vote for 2 weeks in January.  Happy New Year and have a safe holiday.