eMedicine Specialties > Neurology > Movement and Neurodegenerative Diseases
Striatonigral Degeneration: Treatment & Medication
Updated: Mar 9, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
Treatment
Medical Care
Response to anti-parkinsonian medications is sub-optimal at best for treatment of multiple system atrophy; however, it remains the treatment of choice in the absence of better options. Other medical therapies used in multiple system atrophy target associated symptoms (eg, orthostatic hypotension). See Medication and In/Out Patient Meds below.
Surgical Care
Currently, no surgical treatment is appropriate for multiple system atrophy. Because it can be difficult to clinically distinguish multiple system atrophy from Parkinson disease, there are cases of multiple system atrophy patients undergoing placement of deep brain stimulators. The outcomes have generally been poor, even in patients who responded well to levodopa therapy.11
Consultations
- Neurology
- Sleep medicine
- Otolaryngology
- Physical and occupational therapy
- Speech-language pathology
- Psychiatry or counseling
Diet
Unless there are contraindications, patients with symptomatic postural hypotension may benefit from increased salt intake.
Activity
Patients with symptomatic postural hypotension should be advised to avoid activities or environments that produce excessive vagal stimulation or vasodilation (eg, extreme heat, overeating, straining at stool) and to rise slowly and carefully from seated or recumbent positions.
Medication
The drugs in the tables below are specific to treatment of parkinsonism and postural hypotension associated with striatonigral degeneration (multiple system atrophy with predominantly parkinsonian features [MSA-P]). See In/Out Patient Meds for a discussion of adjunct medications.
Antiparkinson agents
Dopaminergic drugs can exacerbate orthostatic hypotension. They must be initiated at low doses and titrated up cautiously.
Levodopa-carbidopa (Sinemet)
Levodopa is a dopamine precursor used to increase CNS dopamine concentration, as it is not possible for dopamine to cross the blood-brain barrier. Carbidopa is a peripheral dopa decarboxylase inhibitor that prevents premature conversion of levodopa to dopamine in the tissues prior to entering the CNS. It increases the efficiency of levodopa therapy, allows for lower dosages, and also decreases the side effects associated with peripheral conversion.
Standard release forms are available in 25/100 mg, 10/100 mg, and 25/250 mg tablets. Controlled-release preparations are available in 50/200 mg and 25/100 mg.
Adult
25/100 mg (standard release form) PO tid initially; may titrate upward using a combination of dosage and frequency changes
Carbidopa 70-100 mg PO qd required to completely block peripheral dopa decarboxylase and minimize nausea/vomiting
Pediatric
Not established
Hydantoins, pyridoxine, phenothiazine, and hypotensive agents may decrease effects of levodopa; antacids and MAOIs increase levodopa toxicity
Documented hypersensitivity, narrow-angle glaucoma, malignant melanoma or undiagnosed skin lesions, non-selective MAOI use within 2 wk
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in patients with history of cardiac disease, arrhythmia, asthma or other severe pulmonary disease, peptic ulcer disease, endocrine disorders, diabetes (may worsen glucose control), anti-hypertensive therapy (may exacerbate postural hypotension), chronic wide-angle glaucoma, renal insufficiency, depression or psychosis (may increase risk of suicidal ideation); risk of neuroleptic malignant syndrome with abrupt discontinuation of medication
Pramipexole (Mirapex)
Non-ergot dopamine agonist, used with or without concomitant levodopa therapy. Binds D2 and D3 dopamine receptors. Due to high specificity for D3 receptors (relative to other dopamine agonists), it may cause less orthostatic hypotension. It has no significant effect on other adrenergic or serotonergic receptors. Absolute bioavailability is >90%. Peak serum concentration is reached in approximately 2 h and half-life is approximately 8 h. There are no known metabolites; roughly 90% of this drug is renally-excreted in its unchanged form.
Tablets are available in 0.125 mg, 0.25 mg, 0.5 mg, 0.75 mg, 1 mg and 1.5 mg forms.
Adult
0.125 mg PO tid initially; may titrate up as tolerated to maximum of 1.5 mg PO tid; when using pramipexole concomitantly with levodopa, the dose of latter can be reduced; dose adjustments are required for patients with renal insufficiency
Pediatric
Not established
Cimetidine may increase toxicity (increases levodopa levels)
Documented hypersensitivity, breastfeeding
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
May worsen existing orthostatic hypotension; caution advised for patients with renal insufficiency (dose adjustment required); caution when driving or operating machinery (may cause somnolence); may exacerbate underlying psychoses; avoid abrupt withdrawal due to risk of neuroleptic malignant syndrome
Ropinirole (Requip)
Non-ergot dopamine agonist, used with or without concomitant levodopa therapy. Binds to D2 and D3 receptors, with greater affinity for D3. Bioavailability is 55%; peak plasma concentration is reached in 1-2 h and half-life is approximately 6 h. It is extensively metabolized by the liver via P450 CYP1A2. Less than 10% renally excreted; no dosage change required in mild to moderate renal insufficiency. If using as adjunct therapy, may be able to titrate levodopa dosage slowly downward.
Adult
0.25 mg PO tid initially; titrate gradually upward as tolerated to maximum dose of 24 mg/d (titrate slowly in patients with hepatic impairment)
Pediatric
Not established
Antipsychotics may antagonize effects of ropinirole; antiemetics such as metoclopramide may also decrease effectiveness; amiodarone, cimetidine, ciprofloxacin and other P450 CYP1A2 inhibitors may lead to increased plasma concentrations; carbamazepine, phenobarbital, rifampin and other P450 CYP1A2 inducers may conversely lower plasma levels; estrogens reduce clearance; concomitant use of CNS depressants may potentiate sedative effects; for patients on warfarin, closely monitor INR, dosage adjustment may be required
Documented hypersensitivity, significant orthostatic hypotension, syncope, pregnancy, breastfeeding
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Monitor carefully for signs and symptoms of orthostatic hypotension or dyskinesia; caution in patients with hepatic disease; patients should not drive or operate heavy machinery (due to side effect of somnolence); this drug may exacerbate pre-existing psychoses; avoid abrupt discontinuation
Mineralocorticoids
For treatment of orthostatic hypotension refractory to nonpharmacologic recommendations.
Fludrocortisone (Florinef)
Synthetic steroid with predominantly mineralocorticoid activity. Acts on renal distal tubules to enhance reabsorption of sodium and increase urinary excretion of potassium. The net effect is an increase in plasma volume and elevation of blood pressure. Metabolism is primarily hepatic.
Adult
0.1-0.2 mg PO bid/tid; dosages vary among individuals and must be tailored for each patient
Pediatric
Not established
Drugs that affect potassium balance may cause or exacerbate hypokalemia; use with fluoroquinolones increases risk of tendon rupture; anti-epileptic medications may decrease effectiveness of this drug; steroid use when vaccines are administered may lead to inadequate immune response
Documented hypersensitivity, systemic fungal infections
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Use caution in patients with heart failure or other cardiac disease, endocrine conditions, electrolyte abnormalities, hepatic failure, diabetes, osteoporosis and GI disorders; monitor electrolytes and glucose during use and taper dose gradually to discontinue
Sympathomimetic agents
For treatment of orthostatic hypotension refractory to non-pharmacologic recommendations.
Midodrine (ProAmatine)
Selective alpha1-adrenergic agonist used for treatment of hypotension.
Adult
10 mg PO tid at 3-4h intervals, during daytime hours when patient is upright; not for administering after evening meal or less than 4 h before bedtime; midodrine is also available in IV form but is most commonly administered orally; dose adjustment is required for patients with renal insufficiency
Pediatric
Not established
Drugs that stimulate alpha-adrenergic receptors may enhance or potentiate pressor effects (this includes over-the-counter cold remedies and diet aids); certain cardiac medications, (eg, glycosides, beta-blockers) may precipitate or worsen bradycardia, AV block, and arrhythmia; concomitant use of tricyclic antidepressants augments vasoconstriction and other alpha-adrenergic effects which can lead to hypertension, tachycardia, or arrhythmia; drugs such as doxazosin or terazosin directly antagonize alpha-adrenergic effects of midodrine; concomitant use with dihydroergotamine is contraindicated due to potential for severe vasoconstriction
Documented hypersensitivity, acute renal insufficiency, severe organic heart disease, pheochromocytoma, urinary retention, persistent and excessive supine hypertension, thyrotoxicosis, concomitant use with dihydroergotamine
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in cardiovascular disease; monitor for bradycardia or elevated supine blood pressure; dose adjustment and careful monitoring required for patients with renal insufficiency; midodrine can cause or exacerbate pre-existing urinary retention; CNS effects reported by some patients include irritability, excitability, and restlessness
More on Striatonigral Degeneration |
| Overview: Striatonigral Degeneration |
| Differential Diagnoses & Workup: Striatonigral Degeneration |
 Treatment & Medication: Striatonigral Degeneration |
| Follow-up: Striatonigral Degeneration |
| References |
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References
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Further Reading
Keywords
striatonigral degeneration, SND, neurodegenerative disease, multiple system atrophy, MSA, MSA-P, Shy-Drager syndrome, sporadic olivopontocerebellar degeneration, sporadic OPCA, sOPCA, parkinsonism, MSA with predominantly parkinsonian features, MSA with predominantly cerebellar features, MSA-C, parkinsonian MSA, cerebellar MSA, parkinsonian multiple system atrophy, cerebellar multiple system atrophy
