Frail
Legacy Name: Frail

Neurocardiogenic / Vasovagal Syncope
Convulsive Fainting
Onset: 16 mo. old
Diagnosed: 8 years old
Status: Ongoing

Idiopathic Hypersomnia
Long Sleeping
Onset: 20 years old
Problems with sleep recorded since infancy; insomnia due to anxiety recorded at age 11
Diagnosed: 25 years old
Status: Ongoing / Managed by medication

I plan aspects of my life around my syncope. Because my triggers are pain or perceived pain, I am careful about activities I partake in, what movies I watch, and my involvement when others are hurt. I am careful not to be outside in heat too long, particularly if I have to stand. I try not to stand in lines for long periods of time. I sit on chairs cross-legged or with my knees up to avoid myself being too upright. I sit down when I take hot showers. I carry a water bottle with me at all times. I eat a lot of salty foods. If I need to get my blood drawn or need to get a vaccination, I make sure I have someone who can accompany me to the appointment so that I don't have to drive and so that they can advocate for me if I faint.

Because of my syncope, I also have poor circulation. I get cold easily and it is hard to warm up... and then when I do warm up, I'm too hot. This is because neurocardiogenic syncope is a form of dysautonomia, a term used for a collection of conditions where the autonomic nervous system (ANS) doesn't function correctly. The ANS is responsible for things that your body does automatically (heart beating, regulating blood pressure, breathing, regulating temperature correctly, etc.). I try to dress in layers and often include warm socks and compression gloves to help keep my extremities warm.

My idiopathic hypersomnia makes me tired all the time. I am never not tired. I can always go to sleep. My body has a 12-hour "biological night", so it feels sleep deprived all the time (even though I make sure to get 9-10 hours of sleep every night). I rely on medication (stimulants) to keep me functioning. Without them, I have been known to sleep upwards of 36 hours straight. Even with them, I feel fatigued. Because of my hypersomnia, I am careful to maintain a consistent sleep schedule. I rarely drink alcohol (it affects my ability to wake up the next morning). I try to plan my days in a way that I'm not over-exerting myself.

The Spoon Theory explains well what it feels like to have a chronic condition that affects your energy. It's why I collect spoons in "My Treasure Chest" gallery, and why you might occasionally hear me saying that I'm too "low on spoons" to do something. Please read it if you know anyone with a chronic condition.

The patient is an 18 month old female who was recently seen in our office for her 18 month exam. The patient was well at that time aside from a fractured clavicle. The patient returned today doing well and it was decided to administer her 18 month set of vaccines. Approximately 5-7 minutes after administering the vaccine, the patient suddenly went limp and pale in her mother's arms. I examined her within seconds. The patient had a heart rate and respiratory rate at that time and started a slight cry but appeared still limp and pale. Over the next few minutes her color improved but she became very sleepy. The patient aroused to cry, but would fall asleep again. Approximately 45 minutes later the patient was awake for approximately 5 minutes playing with a toy in our waiting room, also seemed to be responsively pointing to different toys and objects of her clothing at which point mother stood her on her leg and soon after that, the child had another limp episode with paleness again with heart rate and respiratory rate present. Because of these two episodes, it was felt that the patient needed to be watched more carefully in the hospital with monitoring.

The twenty-two month old patient was seen in follow up of her passing out episodes. As you recall she was discharged after a brief hospitalization in the first week of October. There were no further spells until October 31st when she had 2 additional episodes. Both occurred with marked pallor and limpness. The first occurred shortly after she had fallen down and had been crying for several minutes. She was briefly tired after the spells, but was able to resume normal activities shortly. Her mother reports that she had syncopal episodes as an infant.

I had the pleasure of seeing your patient today in consultation in the Comprehensive Epilepsy Clinic. As you know, she is now a 4-year-old girl with a history of numerous “passing out” episodes that began over two years ago. At the age of 21 months, she had her first “seizure.” It was characterized by spontaneous, unprecipitated onset, loss of consciousness and alteration of body tone followed by reported postictal grogginess. She had three spells within the first week of presentation. She continued to be followed over the next year or so having a frequency of events that, according to her parents, occurred in clusters averaging two to three episodes per month. Subsequent episodes seemed to take on a slightly different character and, more often than not, by history were preceded by events associated with minor trauma.

I spent some time discussing with the patient’s parents the possible diagnoses associated with her events. She may well have complex partial seizures especially with the history of intermittent abdominal pain and an occasional appearance of a warning associated with the events. In that case, as you know, having a normal electroencephalogram would not be significantly surprising. The fact that more than half of the events have been preceded by some sort of minor trauma is somewhat concerning although by no means have these events been classic for pallid breath-holding spells. At this time, we can continue to observe her closely, keeping in mind that she may in fact be having simple and/or partial seizures. At this time, the events do not appear to be changing or altering her social interactions; however, her mother does report that since the one event that occurred in the gymnastics class, the patient has refused to return to the class and states that the children think she is “stupid” because of the seizure.

I will plan to see them in follow-up in approximately six months’ time, but have given them my number and instructions to call at any time should further episodes arise.

We had the pleasure to evaluate your 7-year-old patient today. She was accompanied by her parents for the concern of seizure disorder.

We will briefly summarize her history for her our records. Her seizures started at 21 months of age, and her seizures were manifested by eyes rolling back followed by facial color changes with blue, sweating, sometime eye fluttering, facial flushing, and urination. Her seizures were usually brief, but she felt tired after the seizures. Her seizures were in cluster at the first time, but this pattern was not persisted. Her parents noticed about 70% of her seizures were related to minor head trauma. She feels throat tightness and stomachache coming together as an aura, and she experienced many of isolated aura. She has had about 40 to 50 seizures, and once experienced 10 months of seizure free period, but she has had 4 seizures in the last year. Her EEG’s were normal, but her 2 brain MRI’s showed abnormal findings with multiple areas of high signal intensity changes on posterior periventricular region. She has never been on anticonvulsant medication because of concerning side effects and brief nature of her usual seizure. Her recent seizure frequency was once in 3-4 months, but she felt isolated auras once or twice a week.

We discussed with her parents that anticonvulsant medication is required for her situation because of its recurrent nature and her increasing awareness of social relationship with her age.

I had the opportunity today to re-evaluate your 8-year-old patient in the Epilepsy Program. She is being evaluated for convulsive syncope. The frequency of her attacks is approximately four to eight per year. I would like to review fort you the typical time sequence of an attack. Almost always the event is initiated by an unpleasant painful surprising episode. Examples of these are immediately after venipuncture, following a gerbil bite, a cute eye, biting the tongue, scraped knee, sight of blood, scrape on the belly, and so forth. Sometimes the patient will experience such unpleasant events and not go into an attack, but, when they do occur, the next experience she has is a “bad dizzy feeling” in her stomach. Often at that time she will try to get into the bathroom because she knows what is going to happen. Within about 30 to 60 seconds, she basically appears to faint. All of these events have occurred while sitting or standing and never while lying. The initial phase of this is mild rigidity. This is followed by limpness and pallor and the entire event lasts about 45 seconds. She consistently has urinary incontinence for these. Whereas in the past these would make her quite tired and she might sleep for hours, more recently she has weathered them much better and can basically go about her business afterwards.

There have been only a handful of episodes and none recently in which there is no obvious provocation. In one circumstance she was standing for a long time in a line at a store in the summer and simply appeared to pass out. On another occasion, she was walking home towards her mother and passed out while walking. As you know, she has had EEG examinations in the past which have been entirely normal and specifically lacking any epileptiform activity. Upon my last evaluation, I decided to conduct a trial use of an anticonvulsant, thinking it may abort the “convulsive” phase of her syncope, but this was not successful.

It appears to me that the patient has convulsive syncope. I do not think that she has a seizure disorder and recommended that we discontinue the anticonvulsant. I suggest she be evaluated for vasodepressor syncope. In the meantime, we can try to just push her fluids, including a quart a day of Gatorade.

Tilt table test is abnormal with 11.4 second cardiac pause.

I saw your patient today in consultation for her syncopal episodes. As you know, she is a 17-year-old female. She was accompanied by both her parents.

She has previously been treated with salt tablets and corticosteroids. The corticosteroid resulted in elevated blood pressure and was discontinued. She is not compliant with salt tablets. She is compliant with increasing her fluid intake, according to her parents. Two years ago she had two spells: one occurred in biology class, another occurred in her physician’s office when she was being evaluated for ear pain.

In the clinic today, as we were discussing her symptoms, she had a typical episode. She states that she could feel it coming on. She got to the floor immediately and elevated her legs. I was able to listen to her heart during this and she had a regular rate and rhythm. I checked her blood pressure. It was 80 mmHg systolic. As she continued to feel better, her blood pressure rose to 100 mmHg and her heart rate decreased. She did not lose consciousness, nor did she have loss of her pulse.

My impression is that the parent has a significant component of her episode due to neurocardiogenic syncope. The observed episode today was quite informative. Her response to a discussion of a painful experience is vasodepressor in nature.

We had a long, detailed discussion about therapies. My first recommendation is that she increase salt and fluid intake. I also recommended that she consider support hose as they will also help counteract venous pooling that can occur.

I would like to repeat her tilt table test to look at her heart rate and blood pressure response to upright posture.

Tilt table test aborted. Fainted twice after IV start. Cardiac pauses 18 seconds and 22 seconds in length.

I saw the patient today in follow-up for her neurocardiogenic syncope.

Patient has syncope with negative EEGs, negative MRI, normal echo on 3/1/06 and tilt table test in April 2006 notable for multiple pauses greater than 10 seconds. She was not actually tilted. Started on SSRI and began using applied tension therapy.

Patient has not had any syncopal episode for seven months. I congratulated her on her progress with her therapy. She is clearly improved dramatically.

The patient is a 25 year old female whom I last saw 1/8/14, followed for vasovagal syncope. She has a very complicated history. Detailed review of records from multiple institutions has disclosed the following:

The patient developed convulsive episodes around the age of 2. Periatric evaluation noted no delta wave on ECG. She has a positive tilt table test in 1997, multiple negative EEGs and brain MRIs. Echo on 3/1/06 demonstrated normal biventricular function. Event monitoring at that time ruled out any tacky or brady arrhythmias. A tile table test was scheduled for April 2006, but with starting the IV, she developed multiple 10+ second pauses and she was not actually tilted. She was subsequently started on an SSRI with recommendation for increased salt and fluid intake with good effect. She was on the SSRI for about 6 months, and was stopped for unclear reasons. This was resume in 2010. Between 2010-2013 she did well, until she went to the Marshall Islands to teach, where she had 3 episodes of syncope, as well as an episode in October 2013.

Other past history is notable for depression, anxiety, OCD, and trouble sleeping.

After her episode of syncope in October 2013, she was seen by neurology, who noted that recent workup has included brain MIR (8/9/13, normal). EEG on 11/6/13 showed no epileptiform activity. It was felt that she had convulsive syncope.

She has fainted twice since her last visit with me. One was after a blood draw in February 2014. Next was in early May 2014 at Panera. She had not eaten that much in the morning, but when the EMTs came, her blood sugar was normal.

I have encouraged regular meals, salt, and hydration, to re-attempt compression stockings, and to begin gradual exercise.

Tilt table test results.

Impression: This was an abnormal study. This study provides evidence of a predisposition to neurally-mediated syncope. Measures of parasympathetic, sympathetic adrenergic and sympathetic cholinergic function were in the normal range. This study provides no evidence of orthostatic hypotension. There was no evidence of an exaggerated postural tachycardia.

There was a hypotensive-bradycardic response consistent with neurally-mediated syncope during the tilt table test. The lowest recorded blood pressure was 73/50 mmHg from a baseline of 121/82 mmHg after 11 minutes on the tilt table test. This was accompanied by an abrupt drop in heart rate followed by asystole.