On first evaluation, discontinue anticholinergics or other medications that impair attention and memory. Discontinue any medications that may cause parkinsonism. Start vitamin E if the patient has memory loss. Consider empiric treatment of depression and initiate a trial of levodopa/carbidopa (Sinemet) if rigidity and movement disorder are disabling. Institute a plan for titration of this medication to an appropriate level before declaring the patient to be a "levodopa/carbidopa failure." Consider botulinum toxin injections if the patient has painful limb dystonia. Obtain an EEG if the patient has polymyoclonus or rapid decline. Refer to occupational, physical, and speech therapists, as needed, for gait and safety evaluation, assistive devices, and an exercise program to maintain endurance and strength.
On second evaluation, treat any systemic conditions identified on serologic testing. Discontinue Sinemet if ineffective, and begin empiric trial of second- or third-line dopaminergic agent or consider treatment with clonazepam for myoclonus. Consider a spinal tap if any symptoms suggestive of CNS Whipple disease are present; discuss this possibility with the patient and family. Refer the patient to a geriatric nurse practitioner, case manager, or other dementia resource persons when available. Share reading material on CBGD and dementia with the patient and family. Coordinate consultation with a behavioral neurologist or movement disorder specialist if the family desires.
On third evaluation, treat any systemic conditions further identified, perform spinal tap, and consider brain biopsy if the diagnosis is still in doubt or if the family or patient may benefit. Consider further adjustment of dopaminergic therapies depending upon clinical response.
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This illness is frustrating to patients, their families, and the physicians who care for them. Since insight and memory tends to be preserved throughout most of their illness, depression is common and should be treated when it evolves. Physical, occupational, and speech therapy can be helpful although as the illness progresses third party payers tend to not reimburse for these services, unfortunately. Medications provide little benefit, but agents such as Sinemet are worth trying. All sleep disorders such as sleep apnea and restless legs syndrome should be evaluated and treated as improvement in quality of life for patients and their loved ones can occur. I
realize I have not painted a pleasant picture to those suffering
from this illness and their loved ones, but I must be honest in
what research thus far has taught us. The frustration over
misdiagnosis is problematic for patients and families as many are
diagnosed with Parkinson’s disease or a stroke. Misdiagnosis
for clinicians and researchers adds to the confusion regarding
CBD, as our research has recently shown that only half of those
diagnosed in life with CBD are actually found to have CBD when
brain tissue is examined after death (by autopsy). Other disorders
that can appear identical to CBD during life (also known as CBD
mimickers) include Alzheimer’s disease (AD), Pick’s
disease, progressive supranuclear palsy (PSP), nonspecific
degenerative changes, and rarely Creutzfeldt-Jakob disease. Thus a
definitive diagnosis of CBD requires examination of tissue after
death. The high misdiagnosis rate makes research on patients
suffering from presumed CBD during life difficult to
interpret. A
similar approach is being applied to tau-related disorders. There
are members of several families around the world who have
developed what is called “frontotemporal dementia and
parkinsonism” or FTDP, in which abnormal tau is found in the
brain tissue of those who have granted permission for autopsy. The
findings are quite similar to CBD. Scientists from various
institutions around the world pooled their efforts and identified
several mutations in the tau gene that cause this illness (as of
early 2000, there is no genetic test available for clinical use,
but this may become available in the future). Clearly, there are
other genetic and probably environmental factors involved in the
pathogenesis of CBD, but strains of mice carrying the abnormal tau
gene are being developed, and research with a variety of medicines
will begin soon. Most researchers are quite optimistic that
preventative and/or disease-altering medicines will be developed,
but when this will occur, what side-effects will be present with
treatment, and how costly treatment will be, are not yet
known. Thanks to all who strive to optimize quality of life for those confronting this illness. Brad Boeve, MD |
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.
A benzodiazepine drug having anxiolytic, anticonvulsant, muscle relaxant, and hypnotic properties.[1] It is marketed by Roche under the trade nAame Klonopin in the United States and Rivotril in Australia, Brazil, Canada and Europe. Other names such as Ravotril, Rivatril, Clonex, Paxam, or Kriadex are known throughout the rest of the world.[citation needed] Clonazepam has an unusually long half-life of 18–50 hours, making it generally considered to be among the long-acting benzodiazepines