Cortical Basal Ganglionic Degeneration Medication
- Author: A M Barrett, MD; Chief Editor: Selim R Benbadis, MD more...
Unfortunately, no treatment trials of medication for CBGD have been completed to date; thus, no regimen is reported to be highly effective in slowing or reversing its motor or cognitive symptoms. In primary progressive aphasia, cholinesterase inhibitors can be tried; these have been reported to benefit patients with other tauopathies. Medications for Parkinson disease, including anticholinergics, levodopa, and dopamine agonists, may improve symptoms to some extent in many patients and usually are tried at some point during the course of the disease.
These agents are dopamine receptor agonists.
This agent, approved for the treatment of Alzheimer disease in the US, has both dopaminergic and neuroprotective properties. N-methyl-D-aspartate (NMDA) antagonist. Although no published evidence can be currently identified to support its use in CBGD, theoretically this agent might slow the progression of the disorder, or improve motor function.
Unresponsiveness to this medication supports diagnosis of CBGD; thus, an empiric trial, titrated to high dose (many advocate minimum 4 g daily), is recommended in every patient.
Semisynthetic ergot alkaloid derivative; strong dopamine D2-receptor agonist; partial dopamine D1-receptor agonist. Stimulates dopamine receptors in corpus striatum.
Approximately 28% absorbed from GI tract and metabolized in liver. Approximate elimination half-life is 50 h with 85% excreted in feces and 3-6% eliminated in urine.
Initiate at low dosage; slowly increase dosage to individualize therapy. Maintain levodopa dosage during introductory period.
Assess dosage titration every 2 wk. Gradually reduce dose in 2.5-mg decrements if severe adverse reactions occur.
Often not helpful but a trial probably worthwhile for patients with disabling rigidity.
Nonergot dopamine agonist with specificity to D2 dopamine receptor but has also been shown to bind to D3 and D4 receptors and may stimulate dopamine activity on nerves of striatum and substantia nigra. Often not very helpful, but trial worthwhile.
Unknown mechanism of action; may release dopamine from dopaminergic terminals.
No studies demonstrate that therapy with neuroprotective drugs slows the course of CBGD. However, such therapy does affect the course of other neurodegenerative dementias; therefore, neuroprotective agents generally are offered empirically.
Might protect polyunsaturated fatty acids in membranes from attack by free radicals.
Nonsteroidal anti-inflammatory agents (NSAIDS)
Have analgesic, anti-inflammatory, and antipyretic activities. Their mechanism of action is not known, but they may inhibit cyclooxygenase activity and prostaglandin synthesis. Other mechanisms may exist as well, such as inhibition of leukotriene synthesis, lysosomal enzyme release, lipoxygenase activity, neutrophil aggregation, and various cell-membrane functions.
Numerous studies suggest neuroprotective effect in preventing or slowing course of dementia of Alzheimer type.
Useful for the management of myoclonus. By binding to specific receptor sites, these agents appear to potentiate the effects of GABA and facilitate inhibitory GABA neurotransmission and other inhibitory transmitters.
Reduced disabling myoclonus in 23% patients in one trial. Suppresses muscle contractions by facilitating inhibitory GABA neurotransmission and other inhibitory transmitters.
These may be useful for the management of dystonia, especially painful dystonia. Botulinum toxin can inhibit transmission of impulses in neuromuscular tissue.
Useful in reducing excessive, abnormal muscular contractions. Binds to receptor sites on motor nerve terminals and after uptake inhibits release of acetylcholine, blocking transmission of impulses in neuromuscular tissue.
Re-examine patients 7-14 d after administering initial dose to assess for satisfactory response.
Increase doses twofold over previously administered dose for patients who experience incomplete paralysis of target muscle. Doses of 200-300 units usually administered; maximum safe dose believed to be 400 units.
Galantamine may reduce symptoms of primary progressive aphasia in cortical basal ganglionic degeneration. Other cholinesterase inhibitors may also be effective, although supportive data are preliminary and only available for galatamine at this time.
Galantamine is a competitive and reversible inhibitor of acetylcholinesterase. While the mechanism of action us unknown, it may reversibly inhibit cholinesterase, which may, in turn, increase concentrations of acetylcholinesterase available for synaptic transmission in the CNS and enhance cholinergic function. There is no evidence that acetylcholinesterase inhibitors alter the course of underlying dementia.
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