CHIARI MALFORMATION AND ITS IMPACT ON COGNITIVE FUNCTION AND SENSORY PROCESSING Leslie Schrader, Jill Fraizer No two people with Chiari exhibit the exact same set of symptoms, yet, a significant amount of patients experience symptoms that are the same. Each Chiarian surveyed within this report has a minimum of five symptoms still lingering even many years after their last decompression surgery, with some experiencing more symptoms than before surgery. Repeatedly, we are told that certain symptoms are not related to Chiari malformation. We would be willing to accept that as a fact if our other common denominator could be found. Unfortunately, at this point, sufficient research has not been done on this disorder nor has there been enough taught on Chiari malformation in medical schools. Very few have had the proper training to qualify them as experts. We need more research and education.
CHIARI MALFORMATION AND ITS IMPACT ON COGNITIVE FUNCTION AND SENSORY PROCESSING INTRODUCTION Many people with Chiari malformation experience memory, sensory perception, cognitive and motor skill deficits, yet many of our medical professionals do not recognize these symptoms as being related to Chiari. Chiari patients are repeatedly told that sensory perception deficits, cognitive functions, especially memory, and language skills most likely have no correlation with their disorder. Even though the cerebellum is not where commands may be initiated, it assists in making these motor commands smoother and more accurate, including cognitive functions (James Knierim). Because we have access to more Chiari patients than a typical medical provider has, we wish to provide them with results that support the fact that more people suffer from these deficits than those who do not. We hope these results emphasize the fact that much more research needs to be done specifically on how the damage to the cerebellum in a patient with Chiari malformation has on cognition, memory, sensory perception and motor skills. METHOD USED A questionnaire was circulated amongst many Chiari populations from around the world via the internet consisting of 80 questions pertaining to cognitive, sensory perception, muscle function, autonomic, balance functions and other symptoms. Fifty three questionnaires were returned. It is possible that those who would have responded that they feel no residual or long-lasting Chiari symptoms did not participate in this study because they may no longer be a part of an online support community. RESULTS Twenty five percent of respondents (13) have not yet been decompressed, so we can expect that the majority of them will still experience significant symptomatology. However, one would also expect a decrease in symptom- 2
atology after foramen magnum decompression. When asked if they felt they were better post-operatively, 51% of respondents felt they still have some residual effects hanging on. Twelve patients either do not notice any difference after their surgery or feel they are much worse than before with one of those being relieved of initial symptoms, but experiencing an entirely new set. Not one respondent stated they felt their surgery corrected every symptom of Chiari. Not surprisingly, the symptoms that are most commonly associated with Chiari malformation ranked extremely high in the questionnaire among both decompressed and those not decompressed. Many physicians will agree that when a patient presents with typical symptoms such as headache and neck pain that increase in intensity with exertional activities like laughing, coughing, sneezing most likely accompanied by numbness and tingling in the extremities (Jeffrey Greenfield) that these may definitely be related to Chiari (Figure 1). However, what is surprising is that once we removed all of the obvious, anatomically-consistent symptoms, we were left with an extraordinary amount of sensory perception symptoms ranking extremely high as well as cognitive functions with fatigue being the top symptom experienced by patients with Chiari malformation. (Figure 2). These data lead one to believe that a large portion of the Chiari population could be diagnosed with a sensory perception disorder, however only one of the respondents has an official diagnosis. 3
Memory loss ranks in eighth position even when placed amongst the acknowledged Chiari symptoms. We can also notice that only nine less people responded positively to difficulty reading and focusing on text, putting cognitive deficits also high in the symptoms experienced by a patient with Chiari. Even those symptoms that came in with only 4% of respondents experiencing still deserve recognition. The fact that two people out of the 53 experience the same symptom should be considered significant and deserving of more re-search and education. Given the small number of respondents to this questionnaire compared to the entire Chiari population, the potential exists that Chiari patients who did not respond to the questionnaire also experience said symptom. That means the potential exists that for every 1,000 people with Chiari, 40 of them may carry the same symptom. Broken down, this is one out of every 25 patients, a very significant portion of the Chiari population. CONCLUSION These data strongly support evidence that a significant number of people with Chiari malformation experience symptoms of cognitive, motor, speech and sensory perception deficits. Much more research needs to be done on how Chiari affects these areas of our brains. 4
QUESTIONNAIRE RESULTS Totals Percentage Number of Respondents 53 53 I have been decompressed and I still have some residual effects hanging on. 27 50.94% I have not been decompressed. 13 24.53% I have been decompressed and I am worse than before. 9 16.98% I have been decompressed and I don t notice any difference. 2 3.77% Other: It helped some but more symptoms occurred 1 1.89% I have been decompressed and everything appears to be fixed 0 0.00% Symptoms Fatigue 51 96.23% Headache 47 88.68% Pressure / pain in the neck 46 86.79% Painful tension in neck 45 84.91% Visual disturbances / loss of vision / spots in vision / double vision / seeing spots or "halos" / nystagmus 43 81.13% Dizziness 42 79.25% Tingling / numbness in the extremities 42 79.25% General imbalance / clumsiness 41 77.36% Memory loss 41 77.36% Back pain 41 77.36% Intolerance to bright light / difficulty adjusting to light change 39 73.58% Intolerance to loud / confusing sounds 39 73.58% Vertigo from position change or sudden standing 38 71.70% Migraines 37 69.81% Insomnia 36 67.92% Popping / cracking sounds in neck or upper back when stretching 35 66.04% Ringing in ears (like the tone heard in a hearing test) 33 62.26% Pressure in ears / ears feel stopped up 33 62.26% Depth perception problems 33 62.26% Difficulty walking on uneven ground / feeling ground under feet 32 60.38% Pressure / pain behind the eyes (soreness in the eyeballs) 32 60.38% Difficulty reading / focusing on text 32 60.38% Burning sensation in extremities /shoulder blades 32 60.38% Tingling / crawling feeling on scalp 31 58.49% 5
Visual floaters 30 56.60% Poor / degraded motor skills 29 54.72% Swaying 29 54.72% Poor blood circulation/cold hands & feet 29 54.72% Spontaneous vertigo 28 52.83% Gag reflex problems / lack of gag reflex 28 52.83% Dehydration / excessive thirst 28 52.83% Restricted movement 27 50.94% Pain when changing position 27 50.94% Nausea 27 50.94% Slurred speech 27 50.94% Dry skin and lips 27 50.94% Hear heartbeat in ears 27 50.94% Pain & tension along ear / eye / jawline 26 49.06% Difficulty negotiating steps 25 47.17% Neck spasms 25 47.17% Difficulty swallowing / lump in throat / sore throat / swollen lymph nodes 25 47.17% Sinus / mucous problems 24 45.28% Frequent urination 24 45.28% Electric like burning sensations 23 43.40% Astigmatism 23 43.40% Decreased sensitivity to temperature 22 41.51% Loss of sexual interest / lack of sensation in pelvic area 22 41.51% Fluid-like sound in ears (like water running) 22 41.51% Legs feel heavy 22 41.51% Decreased sensation to touch in extremities 21 39.62% Hand tremors 20 37.74% "Floating" sensation 20 37.74% Difficulty driving 19 35.85% Skin problems 18 33.96% "Strangling" feeling 18 33.96% Pulling sensation while sitting / standing 16 30.19% Decreased muscle tone 16 30.19% Pressure / tightness in chest 16 30.19% Thinning hair 16 30.19% Menstrual problems /severe cramping during period 15 28.30% Hiccups associated with drinking carbonated beverages 15 28.30% Low body temperature 14 26.42% Sleep apnea 13 24.53% Intense itchiness w/profuse sweating 12 22.64% 6
Loss of bladder control 12 22.64% Throat closes when lying flat 12 22.64% Sudden / abrupt changes in blood pressure due to awkward position of head 11 20.75% Lump in throat 11 20.75% Loss of smell / problems with sense of smell 10 18.87% Drooling 9 16.98% Unequal pupil size 8 15.09% Swollen face 8 15.09% Low blood pressure 8 15.09% Thickening of finger joints 7 13.21% Loss of taste 5 9.43% Oscillopsia 5 9.43% Albinism 2 3.77% Vomit in sleep 2 3.77% Color blindness 1 1.89% Seizures 1 1.89% Black outs 1 1.89% Swelling in back of neck 1 1.89% Weight gain 1 1.89% High temperature, never low 1 1.89% Gastrointestinal issues 1 1.89% Frequent falls 1 1.89% Flushing 1 1.89% Diarrhea/constipation 1 1.89% Mood swings 1 1.89% Subluxations 1 1.89% Dislocations 1 1.89% Pulsing 1 1.89% Related Conditions: Occipital neuralgia 10 18.87% Sleep apnea 9 16.98% Syringomyelia 8 15.09% EDS 6 11.32% Dysautonomia 5 9.43% Scoliosis 5 9.43% Occult tethered cord 4 7.55% POTS 3 5.66% Hydrocephalus 3 5.66% Tethered cord syndrome 3 5.66% Thyroid 3 5.66% 7
Fibromyalgia 3 5.66% Major depressive disorder 2 3.77% Intracranial hypertension 2 3.77% Spina bifida occulta 2 3.77% Cervical instability 2 3.77% Chronic fatigue 2 3.77% GERD 2 3.77% ICP 2 3.77% Seizure disorder 1 1.89% Asthma 1 1.89% Sensory processing disorder 1 1.89% Restless leg syndrome 1 1.89% TMJ 1 1.89% Low blood sugar 1 1.89% High blood pressure 1 1.89% Mast cell activation disorder 1 1.89% TMD 1 1.89% Pseudotumor cerebri 1 1.89% Cervicomedullary syndrome 1 1.89% Kyphotic brain stem 1 1.89% Myofascial pain syndrome 1 1.89% Anxiety 1 1.89% Asthma 1 1.89% Kidney stones 1 1.89% Hyperlipidemia 1 1.89% Hyperkalemia 1 1.89% Osteogenesis imperfecta 1 1.89% Scarlet fever 1 1.89% Rocky Mountain spotted fever 1 1.89% 8
WORKS CITED James Knierim, Ph.D., Department of Neuroscience, The Johns Hopkins University. Overview: Functions of the Cerebellum. n.d. 20 10 2013 <http://neuroscience.uth.tmc.edu/s3/chapter05.html>. Jeffrey Greenfield, Ph.D., M.D. Weill Cornell Brain and Spine Center. November 2012. 28 October 2013 <http://weillcornellbrainandspine.org/condition/chiari-malformation>. 9