Since no therapy yet exists for CBD that affects the neurodegenerative process, management must be tailored toward symptoms. Pharmacotherapy directed toward parkinsonism has been disappointing (29). Levodopa, dopamine agonists, and baclofen tend to have little effect on rigidity, spasticity, bradykinesia, or tremor. However, levodopa should be titrated upward as tolerated to at least 750 mg per day in divided doses on an empty stomach to provide an adequate trial. Some patients have noted significant improvement in parkinsonism but this rarely persists beyond several months. In those who do improve dramatically with levodopa therapy, one must question whether CBD is the underlying disorder, as levodopa-refractory parkinsonism is considered by many to be a characteristic feature of the CBS and CBD. Anticholinergic agents rarely improve dystonia, and their use is limited by side effects. Botulinum toxin can alleviate pain owing to focal dystonia. Central pain has been rare, but some patients have responded to gabapentin. Tremor may respond initially to propranolol or primidone, but their effects wane with progression of the disorder. Clonazepam and/or gabapentin may reduce myoclonus in some cases. Although intuitively one would not consider any of the cho-linesterase inhibitors to be beneficial in this disorder, we have seen rare cases who note improvement in psychomotor speed, concentration, and problem-solving abilities with donepezil, rivastigmine, or galantamine. Vitamin E and other antioxidant agents have been tried in hopes of delaying progression of the disorder, but there is no evidence yet supporting a disease-altering effect.
Because of the poor response to pharmacotherapy, the mainstay of management is therefore physical, occupational, and speech therapies. A home assessment by an occupational therapist can aid in determining which changes could be made to facilitate functional independence (e.g., replacing rotating doorknobs with handles; attaching specially formed pads around the handles of eating utensils and toothbrushes; purchasing clothing with velcro instead of buttons or laces, etc.). Passive range of motion (ROM) exercises minimize development of contractures, and all caregivers should be instructed to provide passive ROM exercises daily. In those who develop dystonic flexion of the hand musculature to form a "fisted hand," the fingernails can become imbedded in the palmar tissue leading to cellulitis and even osteomyelitis of the hand. One can avoid this by clipping the fingernails periodically, and by placing a rolled-up washcloth or hand towel in the palm of the hand. All patients experience gait impairment at some point during their illness. A walker with handbrakes can improve ambulation for some patients. Wheelchair and handicap priviledges are warranted in essentially every patient. Use of a bedside commode is also worthwhile. Apraxia is often the most debilitating feature of the disorder, and this feature can complicate one's ability to operate a wheelchair or motorized scooter. However, many patients are able to learn to operate these devices and use them effectively for months or years. We have seen a few patients whose "useless hand" was "made useful" by employing constraint-induced movement therapy. Some third-party payers have denied coverage for these devices and therapies, which is very unfortunate as any element of functional improvement and independence is important for these patients. Speech therapy and communication devices can optimize communication when dysarthria, apraxia of speech, or aphasia is present. Therapists also counsel patients and families on swallowing maneuvers and food additives to minimize aspiration when dys-phagia occurs. Feeding gastrostomy should be discussed with all patients, although many decide not to undergo this procedure.
Some patients with the CBS develop elements of other focal cortical degeneration syndromes, such that features of frontotemporal dementia (FTD), primary progressive aphasia (PPA), posterior cortical atrophy (PCA), or some combination of these can evolve. Also, patients who present with one of these syndromes can develop features of the CBS. Management of many of these non-CBS syndromes is discussed elsewhere (99).
Other treatable comorbid illnesses must also be considered, most notably infections (e.g., pneumonia and urinary tract infections), psychiatric disorders, and sleep disorders. Although psychotic features rarely occur in the CBS, depression evolves in essentially every patient, likely owing in part to the preserved insight that is also characteristic of the disorder. Sleep disorders such as obstructive sleep apnea, central sleep apnea, restless legs syndrome, periodic limb movement disorder, etc. occur with some frequency in the CBS, and treatment can improve quality of life (100). REM sleep behavior disorder is very rare in the CBS; in fact if it is present, one must suspect some contribution of synucleinopathy pathology (101). Despite the difficulties of manipulating the headgear as part of nasal continuous positive airway pressure (CPAP) therapy owing to the limb apraxia, CPAP therapy for obstructive sleep apnea can be tolerated and used effectively in many patients. Patients and caregivers eventually require assistance in maintaining optimal care, which can be provided either through home health care or in a skilled nursing facility.
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