Parkinson's disease (PD) is a slowly progressive disorder of the central nervous system affecting more than 1.5 million people in the United States. The disease usually presents in the early 60s, and its frequency increases with aging. Early symptoms include slowness of movement, stiffness and resting tremors that usually begin in one limb. Lack of smell, depression and sleep disorders may precede these symptoms. The loss of neurons that produce dopamine, a chemical that allows the proper functioning of motor pathways, explains the motor problems. Pharmacologic approaches include therapies that replace dopamine or stimulate dopamine receptors. Rehabilitation and consideration for surgery are also critical aspects of management.
Progressive supranuclear palsy
Progressive supranuclear palsy (PSP) is the most common of the atypical Parkinsonian disorders. The prevalence of PSP is estimated to be about six out of 100,000. Symptoms include the tendency to fall early in the disease course, decrease in eye movements, stiffness and cognitive disturbances. Patients may respond to some anti-Parkinson medications, but the response is limited. Rehabilitation is critical for gait and balance.
Corticobasal degeneration (CBD), also called Cortical-Basal Ganglionic Degeneration or Cortico-Basal Ganglionic Degeneration (CBGD), is a rare progressive neurological disorder that may have various presentations. Symptoms such as stiffness, cognitive disturbances and the inability to complete motor tasks typically begin on one side of the body and progress to the other side. Like PSP, patients may respond to some anti-Parkinson medications, though benefits typically are lost over time.
Dementia with Lewy bodies
Dementia with Lewy bodies (DLB) is a neurodegenerative disorder that is the second most frequent cause of dementia in elderly adults. In DLB, a decline in cognitive abilities is caused by the unnatural clumping of normal proteins in the brain. Symptoms may begin with visual hallucinations, fluctuating levels of awareness and autonomic disturbances, and motor symptoms are often similar to those of Parkinson’s disease. Some mood and memory drugs are helpful in DLB.
Multiple system atrophy
Multiple system atrophy (MSA) is a rare neurodegenerative disease characterized by a combination of symptoms that affect several body systems. Symptoms of MSA typically include Parkinson-like symptoms but also include impaired function of the autonomic nervous system which regulates involuntary functions such as blood pressure, bowel control and bladder control. Anti-Parkinson medications may be effective but should be used more cautiously due to problems in the control of blood pressure when standing.
Frontotemporal dementia (FTD) affects middle-aged subjects who present with a gradual and progressive change in behavior, cognition and/or language. Previously called “Pick’s disease,” the symptoms of FTD depend on the predominant part of the brain affected.
Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, invariably fatal brain disorder. In CJD, both neurological and neuromuscular degeneration progress rapidly due to changes in a small protein-like substance within the brain matter. Symptoms include forgetfulness, personality change, involuntary jerks (“startle myoclonus”) and balance problems.
Sometimes called multi-infarct dementia, vascular dementia (VaD) is one of the most common types of dementia, second only to Alzheimer's disease. Symptoms may progress in a step-wise manner as more small infarcts (or strokes) occur in the brain. Minimizing risk factors for stroke (i.e. smoking, high blood pressure, high cholesterol) is critical, and some anti-Alzheimer’s medications may help stabilize memory.
Tremor disorders – such as essential tremor
Tremors are rhythmic, oscillating, involuntary movements of a body part. Tremors may predominate with rest or action, and certain positions may enhance or decrease the movements. Essential tremor (ET) is the most common movement disorder, affecting five to 10 million Americans. ET typically involves the head, limbs and voice. Combinations of medications and occupational therapy are effective treatments for ET. Deep brain stimulation is an alternative approach when medical treatment fails.
Dystonia consists of involuntary positions of a certain body part, often with excessive movements and cramping sensations. Dystonia can affect one or more areas of the body. Typical diagnoses include spasmodic torticollis of the neck, blepharospasm of the eyes, writer’s cramp or similar limb dystonia, and other combinations of abnormal postures. Multiple treatments are available, including local injections with botulinum toxin (Botox® or Myobloc®).
Myoclonus is a fast, sudden, involuntary movement. It may arise from medication side effects, secondary to other diseases, or alone. A detailed workup is necessary to try to determine the origin of such movements. Treatments are tailored to suppress the myoclonus and address the underlying cause.
Tics are semi-voluntary outbursts of motor or vocal activity, and they may be as simple as a sniff or twitch of the hand. They can cause significant stress or anxiety, which may subsequently worsen the tics. Tics are commonly seen as part of Tourette’s syndrome with onset in childhood, but they may also appear during the adult years. The clinic provides multiple treatment modalities for tics and Tourette’s syndrome.
Ataxia describes the imbalance of gait and limb movements classically seen with disorders of a part of the brain called the cerebellum. Ataxic conditions may be inherited or acquired. Complete genetics workups and counseling are available from the clinic. Quality of life is often maximized by the use of physical, occupational and speech therapies.
Stiff person syndrome
Stiff person syndrome is a rare neurological disorder that most commonly occurs in young adults. Symptoms include muscle rigidity and spasms that progress from the trunk to the limbs. Treatment involves muscle relaxants and some anti-autoimmune therapies.