How To Manage Autonomic Symptoms in Multiple System Atrophy. Amanda C. Peltier, MD MS Neurology

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1 How To Manage Autonomic Symptoms in Multiple System Atrophy Amanda C. Peltier, MD MS Neurology

2 Disclosures NIH Autonomic Rare Diseases Consortium

3 Checking your blood pressure several times a day is helpful to your doctor. Checking your blood pressure lying down (supine) and standing is more helpful to monitor medications. Check after lying down for 5 minutes, then right after standing and 5 minutes (or as long as you can stand). If you can t stand, check when sitting down. Keep a log and write down (or draw figure) position when checked. Blood Pressure

4 Autonomic failure is a neurodegenerative disorder in MSA, lesions are in central autonomic pathways RVLM CVLM NA NTS Central Autonomic Failure (Multiple System Atrophy, MSA) Heart Arterioles Spinal cord Cell body of preganglionic neuron Preganglionic sympathetic axon Normal Norepinephrine levels Postganglionic axon Collateral ganglion Sympathetic ganglion Courtesy of Gamboa A. Vanderbilt University Medical Center

5 Approach to treat orthostatic hypotension Step 1: Eliminate Things Cause Symptoms to Be Worse Anti-hypertensives during the day Diuretics Prostate Medications Heavy Meals

6 Approach to treat orthostatic hypotension Step 2: Non-medicine Measures Increase Fluid Intake, 16 oz. tap water Avoid standing quickly or standing motionless Abdominal binder or waist-high pressure stockings Avoid supine position during the daytime

7 Hemodynamic effects of 16oz of water Shibao HTN 2005

8 Approach to treat orthostatic hypotension Step 3: Improve Blood Volume Treat anemia even if mild Fludrocortisone mg every day with increased salt intake Add NaCl tablets 1 gram with meals if necessary

9 Treatment of Orthostatic Hypotension In the past 20 years, only two FDA approved drugs PRESSOR AGENTS qmidodrine, α-1 adrenergic agonist. Direct vasoconstrictor In 2010, the FDA requested to remove it from the market because of lack of evidence supporting clinical benefit in post-marketing studies. qdroxidopa, synthetic norepinephrine precursor Long-term efficacy and safety still under investigation. Limited use because of prohibited cost (~$5,000/30-day supply)

10 Approach to Treat Orthostatic Hypotension Step 4: Short acting pressor agents PRN Midodrine mg TID CC Pyridostigmine 30-60mg TID CC Droxidopa TID CC

11 Stepwise Approach to supine htn Step 2: Nonpharmacologic measures Raise the head of the bed up 6-9 inches Rest on a semirecumbent chair with feet on the floor during the day Encourage snack before bedtime Allow minimal alcohol consumption before bedtime

12 Stepwise Approach to supine htn Step 1: Education and Avoidance Instruct patients about OTC medications with pressoreffects Avoid fluid intake at bedtime Avoid using elastic stockings when supine Avoid pressor agents before bedtime

13 Case 1 67 year old pastor with dizziness and constipation for 2 years. Dizziness improves on lying down. His voice has changed over time. He has worsened memory. He does not tolerate the heat. He has increased urinary frequency. He is noted to have decreased facial movement, increased muscle tone and ridgidity without tremor.

14 Factors Altering BP in Severe Dysautonomia 60 ΔSBP mm Hg Food Clonidine Terbutaline CO 2 SBP

15 Approach to treat orthostatic hypotension Step 4: Short acting pressor agents PRN Midodrine mg TID CC Pyridostigmine 30-60mg TID CC Droxidopa TID CC

16 Supine hypertension ~50% of patients with primary autonomic failure exhibit paradoxic supine hypertension SBP 150mmHg or DBP 90mmHg Often goes undetected BP measured in seated position Automated 24hr BP monitoring helpful Increases end organ damage and risk of acute CV events Limits pressor agents Induces nocturnal diureses

17 Stepwise Approach to supine htn Step 3: Pharmacologic Measures Nitrates, transdermal nitroglycerin ( mg/h, removed in the morning) Hydralazine (50mg) Short-acting calcium blocker, nifedipine (30mg) Minoxidil (2.5mg) Clonidine (0.1mg), early in the evening

18 Stomach and Gut Problems in MSA Constipation A bowel regimen is helpful to prevent constipation Medicine to promote moving your bowels: senna, milk of magnesium, dulcolax (bisacodyl), Medicine to soften bowels as needed: Colace (docusate) Enemas, Fiber, Magnesium Citrate, suppositories Diarrhea Immodium, hyoscamine, bentyl (dicyclomine)

19 Bladder Genitourinary Issues in MSA Medicines to relax bladder muscles like oxybutynin (Ditropan), tolteridine (Detrol), Botox injections, etc. May be helpful in early stages. Self-Catheterization to help empty bladder. Permanent suprapubic catheter if needed. Impotence/Erectile dysfunction Medicines to achieve erection like sildenafil (Viagra), tadalafil (Cialis), vardenafil (Levitra) have to be carefully monitored as can worsen low blood pressure.

20 Conclusions Step wise approach is best. Talk to your doctor about medications recommended. Blood pressure monitoring is helpful to manage low and high blood pressures.