Disturbios do Movimento Flashcards
Quais os dados da história e do exame físico devem ser redflags para um parkinsonismo plus?
História: Early speech and gait impairment, exposure to neuroleptics, onset prior to age 40, liver disease, early halucinations, dipoplia and poor or no response to levodopa.
Exame físico: dementia as the first symptom, prominen orthostatic hypotension, proeminente cerebelar signs, imparment of down gaze and symmetric high frequency postural tremor with a prominent kinet component
Quando iniciar as drogas antiparkisonianas?
When to initiate symptomatic therapy: There is a trend toward initiating therapy at the time of diagnosis or early in the course of the disease because patients may have some disability even at an early stage, and there is the possibility that early treatment may preserve beneficial compensatory mechanisms; however, some experts recommend waiting until there is functional disability before initiating therapy.
Qual droga antiparkisoniana devemos utilizar?
What therapy to initiate: Many experts favor starting with an MAO-B inhibitor in mildly affected patients because of the potential for a disease-modifying effect; dopamine agonists for younger patients with functionally significant disability to reduce the risk of motor complications; and levodopa for patients with more advanced disease, the elderly, or those with cognitive impairment.
Drugs commonly used:
A. Carbidopa/levodopa 10/100, 25/100, 25/250 200–1000 mg levodopa/d 2–4 times/day
B. Dopamine agonists: Pramipexole 0.125, 0.25, 0.5, 1.0, 1.5 mg
0.25–1.0 mg tid
C. COMT inhibitors: Entacapone 200 mg 200 mg with each levodopa dose
D. MAO-B Inhibitors: Selegiline 5 mg 5 mg bid Rasagiline 0.5, 1.0 mg 1.0 mg QAM
There are, however, important limitations of levodopa therapy. Acute dopaminergic side effects include nausea, vomiting, and orthostatic hypotension. These are usually transient and can generally be avoided by gradual titration. If they persist, they can be treated with additional doses of a peripheral decarboxylase inhibitor (e.g., carbidopa) or a peripheral dopamine-blocking agent such as domperidon
Como manejar as complicações motoras das drogas antiparkisonianas?
Management of motor complications: Motor complications are typically approached with combination therapy to try and reduce dyskinesia and enhance the “on” time. When medical therapies cannot provide satisfactory control, surgical therapies can be considered.
In general, dopamine agonists do not have comparable efficacy to levodopa. They were initially introduced as adjuncts to levodopa to enhance motor function and reduce “off” time in fluctuating patients. Subsequently, it was shown that dopamine agonists, possibly because they are relatively long-acting, are less prone than levodopa to induce dyskinesia.
Selegiline and rasagiline are relatively selective suicide inhibitors of the MAO-B enzyme. Clinically, MAO-B inhibitors provide modest antiparkinsonian benefits when used as monotherapy in early disease, and reduced “off” time when used as an adjunct to levodopa in patients with motor fluctuations. MAO-B inhibitors are generally safe and well tolerated. They may increase dyskinesia in levodopa-treated patients but this can usually be controlled by down-titrating the dose of levodopa. The DATATOP study showed that selegiline significantly delayed the time until the emergence of disability, necessitating the introduction of levodopa in untreated PD patients. However, it could not be determined whether this was due to a neuroprotective effect that slowed disease progression or a symptomatic effect that merely masked ongoing neurodegeneration. More recently, the ADAGIO study demonstrated that early treatment with rasagiline 1 mg/d but not 2 mg/d provided benefits that could not be achieved with delayed treatment with the same drug, consistent with a disease-modifying effect; however, the long-term significance of these findings is uncertain.
When levodopa is administered with a decarboxylase inhibitor, it is primarily metabolized by catechol-O-methyltransferase (COMT). Inhibitors of COMT increase the elimination half-life of levodopa and enhance its brain availability. Combining levodopa with a COMT inhibitor reduces “off” time and prolongs “on” time in fluctuating patients while enhancing motor scores. Side effects of COMT inhibitors are primarily dopaminergic (nausea, vomiting, increased dyskinesia) and can usually be controlled by down-titrating the dose of levodopa by 20–30%. No benefit of initiating levodopa with a COMT inhibitor compared to levodopa alone was detected in early PD patients in the STRIDE-PD study, and the main value of COMT inhibitors for now continues to be in patients who experience motor fluctuations.
Quais as medidas nao farmacológicas para manejo da D. de Parkinson?
Nonpharmacologic approaches: Interventions such as exercise, education, and support should be considered throughout the course of the disease.
(1) Gait dysfunction with falling is an important cause of disability in PD. Dopaminergic therapies can help patients whose gait is worse in “off” time, but there are currently no specific therapies available. Canes and walkers may become necessary.
(2) Freezing episodes, where patients freeze in place for seconds to minutes, are another cause of falling. Freezing during “off” periods may respond to dopaminergic therapies, but there are no specific treatments for “on” period freezing. Some patients will respond to sensory cues such as marching in place, singing a song, or stepping over an imaginary line.
(3) Exercise, with a full range of active and passive movements, has been shown to improve and maintain function for PD patients. It is less clear that formal physical therapy is necessary, unless there is a specific indication. It is important for patients to maintain social and intellectual activities to the extent possible.
(4) Education, assistance with financial planning, social services, and attention to home safety are important elements of the overall care plan. Information is available through numerous PD foundations and on the web, but should be reviewed with physicians to ensure accuracy. The needs of the caregiver should not be neglected. Caring for a person with PD involves a substantial work effort and there is an increased incidence of depression among caregivers. Support groups for patients and caregivers may be useful.
Quais as complicações psiquiátricas do tratamento da D. de Parkinson?
Behavioral alterations can be encountered in levodopatreated patients. A dopamine dysregulation syndrome has been described where patients have a craving for levodopa and take frequent and unnecessary doses of the drug in an addictive manner. PD patients taking high doses of levodopa can also have purposeless, stereotyped behaviors such as the meaningless assembly and disassembly or collection and sorting of objects. This is known as punding, a term taken from the Swedish description of the meaningless behaviors seen in chronic amphetamine users. Hypersexuality and other impulse-control disorders are occasionally encountered with levodopa, although these are more commonly seen with dopamine agonists. Recently, it has become appreciated that dopamine agonists are associated with impulse-control disorders, including pathologic gambling, hypersexuality, and compulsive eating and shopping
Qual o uso do DBS na doença de Parkinson?
Most surgical procedures for PD performed today utilize deep brain stimulation (DBS). Here, an electrode is placed into the target area and connected to a stimulator inserted SC over the chest wall. DBS simulates the effects of a lesion without necessitating a brain lesion. The stimulation variables can be adjusted with respect to electrode configuration, voltage, frequency, and pulse duration in order to maximize benefit and minimize adverse side effects. In cases with intolerable side effects, stimulation can be stopped and the system removed. The procedure has the advantage that it does not require making a lesion in the brain and is thus suitable for performing bilateral procedures with relative safety. DBS for PD primarily targets the STN or the GPi. It provides dramatic results, particularly with respect to “off” time and dyskinesias, but does not improve features that fail to respond to levodopa and does not prevent the development or progression of nondopaminergic features such as freezing, falling, and dementia. The procedure is thus primarily indicated for patients who suffer disability resulting from levodopa-induced motor complications that cannot be satisfactorily controlled with drug manipulation. Side effects can be seen with respect to the surgical procedure (hemorrhage, infarction, infection), the DBS system (infection, lead break, lead displacement, skin ulceration), or stimulation (ocular and speech abnormalities, muscle twitches, paresthesias, depression, and rarely suicide). Recent studies indicate that benefits following DBS of the STN and GPi are comparable, but that GPi stimulation may be associated with a reduced frequency of depression. While not all PD patients are candidates, the procedure is profoundly beneficial for many. Research studies are currently examining additional targets that might benefit gait dysfunction, depression, and cognitive impairment in PD patients.
Como manejar as manifestações nao motoras da doença de Parkinson?
Levodopa and other dopaminergic drugs can aggravate cognitive function in demented patients and should be stopped or reduced to try and provide a compromise between antiparkinsonian benefit and preserved cognitive function. Drugs are usually discontinued in the following sequence: anticholinergics, amantadine, dopamine agonists, COMT inhibitors, and MAO-B inhibitors. Eventually, patients with cognitive impairment should be managed with the lowest dose of standard levodopa that provides meaningful antiparkinsonian effects and does not aggravate mental function. Anticholinesterase agents such as rivastigmine and donepezil reduce the rate of deterioration of measures of cognitive function in controlled studies and can improve attention. Memantine, an antiglutamatergic agent, may also provide benefit for some PDD patients.
(2) utonomic disturbances are common and frequently require attention. Orthostatic hypotension can be problematic and contribute to falling. Initial treatment should include adding salt to the diet and elevating the head of the bed to prevent overnight sodium natriuresis. Low doses of fludrocortisol (Florinef) or midodrine control most cases. Vasopressin, erythropoietin, and the norepinephrine precursor 3-0-methylDOPS can be used in severe cases. If orthostatic hypotension is prominent in early disease, MSA should be considered. Sexual dysfunction can be helped with sildenafil or tadalafil. Urinary problems, especially in males, should be treated in consultation with a urologist to exclude prostate problems. Anticholinergic agents, such as Ditropan, may be helpful. Constipation can be a very important problem for PD patients. Mild laxatives can be useful, but physicians should first ensure that patients are drinking adequate amounts of fluid and consuming a diet rich in bulk with green leafy vegetables and bran. Agents that promote GI motility can also be helpful.
(3) Sleep disturbances are common in PD patients, with many experiencing fragmented sleep with excess daytime sleepiness. Restless leg syndrome, sleep apnea, and other sleep disorders should be treated as appropriate. REM behavior disorder (RBD) may precede the onset of motor features. This syndrome is composed of violent movements and vocalizations during REM sleep, possibly representing acting out of dreams due to a failure of the normal inhibition of motor movements that typically accompanies REM sleep. Low doses of clonazepam are usually effective in controlling this problem. Consultation with a sleep specialist and polysomnography may be necessary to identify and optimally treat sleep problems.
Quais os tipos de movimentos hipercinéticos?
(1) Tremor Rhythmic oscillation of a body part due to intermittent muscle contractions
(2) Dystonia Involuntary patterned sustained or repeated muscle contractions often associated with twisting movements and abnormal posture
(3) Athetosis Slow, distal, writhing, involuntary movements with a propensity to affect the arms and hands
(4) Chorea Rapid, semipurposeful, graceful, dancelike nonpatterned involuntary movements involving distal or proximal muscle groups
(4) Myoclonus Sudden, brief (<100 ms), jerk-like, arrhythmic muscle twitches (5) Tic Brief, repeated, stereotyped muscle contractions that are often suppressible. Can be simple and involve a single muscle group or complex and affect a range of motor activities
Como tratar distonia?
(1) Wilson’s disease should be ruled out in young patients with dystonia.
(2) Levodopa should be tried in all cases of childhoodonset dystonia to rule out DRD.
(3) Oral baclofen (20–120 mg) may be helpful, but benefits if present are usually modest and side effects of sedation, weakness, and memory loss can be problematic. Intrathecal infusion of baclofen is more likely to be helpful particularly with leg and trunk dystonia, but benefits are frequently not sustained and complications can be serious and include infection, seizures, and coma. Tetrabenazine (the usual starting dose is 12.5 mg/d and the average treating dose is 25–75 mg/d) may be helpful in some patients, but use may be limited by sedation and the development of parkinsonism
(4) Botulinum toxin has become the preferred treatment for patients with focal dystonia, particularly where involvement is limited to small muscle groups such as in blepharospasm, torticollis, and spasmodic dysphonia. No systemic side effects are encountered with the doses typically employed, but benefits are transient and repeat injections are required at 2- to 5-month intervals. Some patients fail to respond after having experienced an initial benefit. This has been attributed to antibody formation, but improper muscle selection, injection technique, and inadequate dose should be excluded.
(5) Surgical therapy is an alternative for patients with severe dystonia who are not responsive to other treatments. Peripheral procedures such as rhizotomy and myotomy were used in the past to treat cervical dystonia, but are now rarely employed. DBS of the pallidum can provide dramatic benefits for patients with primary DYT1 dystonia
(6) Supportive treatments such as physical therapy and education are important and should be a part of the treatment regimen.
Como manejar o status distonico/tempestade distonica?
Physicians should be aware of dystonic storm, a rare but potentially fatal condition that can occur in response to a stress situation such as surgery in patients with preexisting dystonia. It consists of the acute onset of generalized and persistent dystonic contractions that can involve the vocal cords or laryngeal muscles, leading to airway obstruction. Patients may experience rhabdomyolysis with renal failure. Patients should be managed in an ICU with protection of airway if required. Treatment can be instituted with one or a combination of anticholinergics, diphenhydramine, baclofen, benzodiazepines, and dopamine agonists/antagonists. Spasms may be difficult to control, and anesthesia with muscle paralysis may be required.
Quais os pilares do tratamento da doença de Huntington?
Treatment involves a multidisciplinary approach, with medical, neuropsychiatric, social, and genetic counseling for patients and their families
(1) Dopamine-blocking agents may control the chorea. Tetrabenazine has recently been approved for the treatment of chorea in the United States, but it may cause secondary parkinsonism. Neuroleptics are generally not recommended because of their potential to induce other more troubling movement disorders and because HD chorea tends to be self-limited and is usually not disabling
(2) Depression and anxiety can be greater problems, and patients should be treated with appropriate antidepressant and antianxiety drugs and monitored for mania and suicidal ideations. Psychosis can be treated with atypical neuroleptics such as clozapine (50–600 mg/d), quetiapine (50–600 mg/d), and risperidone (2–8 mg/d).
Qual o tratamento da doença de Tourrete?
Patients with mild disease often only require education and (1) counseling (for themselves and family members).
Drug treatment is indicated when the tics are disabling and interfere with quality of life. Therapy is generally initi
ated with the α-agonist (2) clonidine, starting at low doses and gradually increasing the dose and frequency until satisfactory control is achieved. Guanfacine (0.5–2 mg/d) is an α-agonist that is preferred by many clinicians because it only requires once-a-day dosing.
If these agents are not effective, (3) antipsychotics can be employed. Atypical neuroleptics (risperidone, olanzapine, ziprasidone) are preferred as they are thought to be associated with a reduced risk of extrapyramidal side effects
s. (4) Behavioral features, and particularly anxiety and compulsions, can be a disabling feature of TS and should be treated
Qual o manejo da mioclonia?
Treatment primarily consists of (1) treating the underlying condition or removing an offending agent. Pharmacologic therapy involves one or a combination of GABAergic agents such as (2) valproic acid (800–3000 mg/d), piracetam (8–20 g/d), clonazepam (2–15 mg/d), or primidone (500–1000 mg/d). Recent studies suggest that levetiracetam may be particularly effective
