Friday, October 26, 2012

Multiple System Atrophy causes of death

Cardiopulmonary arrest7 (33.3)
 Urinary tract infection5 (23.8)
 Aspiration pneumonia2 (9.5)
 Infectious pneumonia2 (9.5)
 Acute aspiration1 (4.8)
 Wasting syndrome3 (14.3)

"Occurrence of sudden death is a common cause of mortality in MSA and may happen in the early stages while disability remains acceptable."
Citation to: Shimohata, T., Ozawa, T., Nakayama, H., Tomita, M., Shinoda, H. and Nishizawa, M. (2008) Frequency of nocturnal sudden death in patients with multiple system atrophy. J Neurol 255: 1483-1485.
Sudden death has been reported in patients with multiple system atrophy (MSA), although the frequency of this event has not been well delineated. We investigated the frequency and potential causes of sudden death in patients with MSA. During the 5-year observation period, 10 of 45 patients with probable MSA died. The causes of death included sudden death of unknown etiology (seven patients), aspiration pneumonia (one patient), asphyxia after vomiting (one patient), and lung cancer (one patient). The mean survival time of patients with sudden death was 63.0 ± 24.7 months (range, 39–116 months). Among seven patients who experienced sudden death, six were found to have died during sleep. Among these patients, two had been treated with tracheostomy and three with continuous positive airway pressure (CPAP) or noninvasive positive pressure ventilation (NPPV) during sleep, suggesting that these treatments do not always prevent sudden death in patients with MSA. Nocturnal sudden death should be recognized as the most common mechanism of death in patients with MSA.
The close relationship between life-threatening breathing disorders and urine storage dysfunction in multiple system atrophy. K Deguchi, K Ikeda, R Goto, M Tsukaguchi, Y Urai… - Journal of Neurology, 2010 - Springer
Survival of multiple system atrophy (MSA) depends on whether a variety of sleep-related breathing problems as well as autonomic failure (AF) occur. Since the brainstem lesions that cause respiratory and autonomic dysfunction overlap with each other, these critical manifestations might get worse in parallel. If so, the detection of AF, which is comparatively easy, might be predictive of a latent life-threatening breathing disorder. In 15 patients with MSA, we performed autonomic function tests composed of postural challenges and administered a questionnaire on bladder condition, as well as polysomnography and laryngoscopy during wakefulness and under anesthesia. Polysomnographic variables such as the apnea-hypopnea index (AHI) and oxygen saturation (SpO2) and the findings of laryngoscopy were compared with the degree of cardiac and urinary autonomic dysfunction. AHI, mean SpO2 and the lowest SpO2 showed significant correlations with urine storage dysfunction. In addition, patients with vocal cord abductor paralysis (VCAP) or central sleep apnea (CSA) contributing to nocturnal sudden death had more severe storage disorders than those without. On the other hand, no significant relationship between polysomnographic variables and orthostatic hypotension was observed except in the case of mean SpO2. These results indicate that life-threatening breathing disorders have a close relationship with AF, and especially urine storage dysfunction. Therefore, longitudinal assessment of deterioration of the storage function might be useful for predicting the latent progress of VCAP and CSA.

Saturday, October 06, 2012

Cognitive changes in Atypical Parkinson's

I just watched this presentation by an Australian neuropsychiatrist (about 45 minutes), and it is very helpful!

She says adynamia (loss of drive and initiative) is the #1 issue.  For MSA she also talks about rigidity (loss of flexibility of thinking).   For all the atypical Parkinson’s she give a list of features:
  • Reduced insight
  • Mental inflexibility
  • Can’t wait, narrow focus
  • Reduced empathy
  • Lack of appropriate concern (for example about the future progression of the disease)
That's such an accurate list of my frustrations.

Other useful lectures:

Wednesday, October 03, 2012

John's medical history

I compiled this when someone asked and I am putting it here so I won't lose it.

From childhood:
  • Dyslexia (difficulty learning to read and reversals)
  • Difficulties with attention and memory
  • Poor coordination
Early adulthood:
  • Difficulty with learning languages
  • Difficulty with memory and organization
  • Tendency to be late, poor sense of how long things will take
  • A neurological event that was never explained—pain in his chest and a band of numbness around his abdomen that lasted a few weeks
  • Ongoing problems with insomnia
  • Poor sense of smell
  • Probably already had a 20 point gap between verbal and performance IQ—our son does
40s and 50s
  • Diagnosed borderline Attention Deficit Disorder
  • Managed well following a routine but didn’t deal well with change
  • Snoring or sometimes a soft sound breathing in only, when sleeping on his back
  • Problems with constipation
  • Took antacids after every meal (this has continued, even when he went on acid blockers)
  • Swallowing issues diagnosed as Schotsky’s ring and successfully treated
The two or three years before diagnosis at age 62
  • Increasing slowness—set his alarm earlier and earlier to get to work on time
  • Yelling and hitting out in his sleep (once he bit my hand)
  • Began to make mistakes when buying airline tickets
  • Tested as having a little sleep apnea, not enough to need treatment
Age 62—diagnosed by a neurologist with Parkinsonism and then by the Movement Disorder Specialist with Lewy Body Dementia (he didn’t have all the symptoms yet but that doctor thought he was on that path)
  • Cogwheel rigidity, worse on his nondominant side
  • Only swung one arm when walking
  • Could not spell the word WORLD backwards when we met with the MDS
  • He noticed he was making mistakes at work and was able to get disability retirement and Social Security Disability
First few years after diagnosis
  • Head tilted forward
  • Neuropsych testing showed a 30 point gap between verbal and performance IQ
  • Occasional confusion
  • I took over almost all bill paying and household organization—luckily he was always somewhat passive and didn’t get upset about losing control
  • Test for sleep apnea a year after the first test showed less
  • Blood pressure on the low side when tested in the doctor’s office
  • No more heavy sweating
  • Swelling of his lower legs, eventually treated with Velcro-closed elastic wraps
  • Alternating diarrhea and constipation
  • Surgery for benign prostate enlargement resulted in little improvement
  • Passed driving evaluation by a specialist OT at the rehab hospital each year
Last couple of years (age 65-67)
  • Takes him several hours to get ready and shower
  • Serious swelling of his lower legs, finally treated as lymphedema with velcro-closed wraps (which someone has to put on for him)
  • Occasional bowel and bladder incontinence
  • Rapid shallow breathing, but he says he feels no shortness of breath
  • Still able to make his own breakfast and lunch and talk politics with friends
  • Increasingly noticeable that he misreads or misunderstands things
  • More focused on himself, tends to say no to doing things even with our kids
  • Walks with very small steps most of the time
  • Diagnosis changed to MSA because he hasn’t had a steep cognitive decline (or hallucinations) as would be typical with Lewy Body Dementia
His current medications:
  • Sinemet (Carbidopa 25 mg/Levidopa 100 mg) 2 tablets 4 times a day
  • Aricept (Donepezil)10 mg once a day
  • Wellbutrin (Bupropion) 300 mg. once a day.
  • Proscar (Finasteride) 5 mg. one once a day for benign prostate enlargement
  • Flomax (Tamsulosin) .4 mg. once a day for benign prostate enlargement.
  • Clonazepam .5 mg at bedtime for sleep and REM sleep disorder
  • Mirtazapine ½ of mg. before bed for sleep (he used to take Clonidine but was taken off it because it lowers blood pressure
  • Omeprazole 40 mg once a day for acid reflux
  • Amitiza (Lubiprostone) 24 mcg once daily for constipation
  • Famciclovir 500 mg once a day for herpes prevention

Supplements and OTC:
  • MCT oil 1 tbsp a day and Coconut oil 1 ½ teasp a day
  • Vitamin D 2000 IU once a day (plus 2000 in vitamin for a total of 4000)
  • Life Extension 2 per day multivitamin
  • Jarro-Dophilus EPS probiotic one a day and Ultra-Zime digestive enzyme supplement 2/day
  • D-Mannose, 500 mg once a day (to prevent UTIs)
  • 3 mg melatonin one a day
  • Tums (Calcium carbonate) 500 mg. antacid about 12 a day as needed
  • Miralax 1/2 dose once a day
  • Metamucil one tblsp a day