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Biomedical subjects

C H Adler

Publications and source records attributed to C H Adler.

At least 37 records · Page 2Linked to original sources
PubMed2000

Strategies for controlling dystonia. Overview of therapies that may alleviate symptoms.

Dystonia is an involuntary movement disorder characterized by twisting, turning, and posturing. This disorder may affect a single body part or may be more generalized, but the pathophysiology remains unclear. The treatment of choice for most of the focal dystonias is botulinum toxin injections, although oral medications occasionally may be beneficial. Surgical treatment of dystonia may be performed peripherally or centrally but is usually reserved for patients in whom other forms of therapy fail.

Botulinum Toxins, Type A↗
PubMed2000

Effect of hormone variations and other factors on symptom severity in women with dystonia.

OBJECTIVE: To determine if any relationship exists between the severity of symptoms in women with dystonia and female reproductive hormonal variations. PATIENTS AND METHODS: We surveyed 279 women with dystonia seen at Mayo Clinic Scottsdale over a 6-year period (1990-1995), and 204 responded. The women were asked questions regarding their reproductive and menstrual histories and dystonia severity and other questions with an emphasis on possible exacerbating or relieving factors. RESULTS: Although in the majority of women hormonal influences had no consistent effect on dystonia symptom severity, 26 (41.9%) of 62 premenopausal women noted a change in the severity of their dystonic symptoms in relation to the 3 phases of their menstrual cycle. Other factors that exacerbated dystonia included stress and fatigue, while sleep improved symptoms. Pregnancy, menopause, and hormone replacement therapy had no effect on symptoms. CONCLUSIONS: Menstrual cycling may result in subjective worsening of dystonia symptoms in some women with dystonia. Further clinical and physiologic evaluation is indicated in such patients, as they may represent an important subgroup of dystonic patients that might yield some clues to the pathophysiology of dystonia and to improved treatment strategies.

Adult↗
PubMed2000

Pharmacological and surgical options for the treatment of cervical dystonia.

Cervical dystonia (CD) is a condition in which patients experience involuntary and abnormal head movements, such as tilting, twisting, or extension, often accompanied by pain. Although the exact pathologic mechanisms underlying idiopathic CD have not yet been identified, a number of therapeutic strategies have been developed to alleviate the symptoms of this disorder. Oral medications include anticholinergic agents, dopamine receptor antagonists, and GABAmimetic agents. These drugs are employed in a trial-and-error manner and have a low rate of efficacy. Localized therapy using botulinum toxin injections has revolutionized the treatment of CD, providing a high rate of response with a low incidence of side effects. However, as with oral medications, neurotoxin therapy is palliative, not curative, and repeated injections are required. In patients who develop resistance to botulinum toxin therapy and who do not achieve an adequate response to, or are intolerant of, oral medications, surgical approaches are appropriate. Among the options for peripheral surgery, the greatest experience and most consistent results have been achieved with selective dorsal ramisectomy. Recent developments in stereotactic surgery suggest that, for more complex forms of CD or when more widespread dystonia is present, bilateral pallidotomy or globus pallidus deep brain stimulation may be the treatment of choice.

Botulinum Toxins, Type A↗
PubMed1999

Safety and efficacy of NeuroBloc (botulinum toxin type B) in type A-resistant cervical dystonia.

OBJECTIVE: To determine the safety and efficacy of botulinum toxin type B (BoNT/B) in patients with type A-resistant cervical dystonia (CD). BACKGROUND: Local intramuscular injections of BoNT are an effective therapy for CD. After repeated use, some patients become resistant to therapy. BoNT/B, effective in type A toxin-responsive patients, is proposed as an alternative therapy for type A-resistant patients. METHODS: The authors performed a 16-week, double-blind, placebo-controlled trial of BoNT/B in type A-resistant patients with CD. After resistance to therapy was confirmed with the frontalis-type A test, placebo or 10,000 U BoNT/B was administered in a single session into two to four clinically involved muscles. The Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) was the primary efficacy measurement. TWSTRS-Total, three visual analog scales (Patient Global Assessment of Change, Principal Investigator Global Assessment of Change, Patient Analog Pain Assessment), and adverse events were assessed at baseline and weeks 2, 4, 8, 12, and 16. RESULTS: A total of 77 patients participated (38 placebo, 39 active). Improvements in severity, disability, and pain were documented in the BoNT/B-treated group. TWSTRS-Total scores were improved in the BoNT/B-treated group at weeks 4 (p = 0.0001), 8 (p = 0.0002), and 12 (p = 0.0129). All three visual analog scales demonstrated improvements at week 4 (p < 0.0001, 0.0001, and 0.001). A Kaplan-Meier analysis supported a duration of effect of 12 to 16 weeks in the active group. Dry mouth and dysphagia were self-limited adverse effects, reported more commonly in the BoNT/B group. CONCLUSIONS: Botulinum toxin type B (BoNT/B) (NeuroBloc) is safe and efficacious for the management of patients with type A-resistant cervical dystonia with an estimated duration of treatment effect of 12 to 16 weeks.

Adult↗
PubMed1999

Differential diagnosis of Parkinson's disease.

The differential diagnosis of PD includes other neurodegenerative disorders; hereditary disorders; and symptomatic causes, such as structural lesions, infections, metabolic abnormalities, hydrocephalus, and drugs or toxins. A good history of symptom evaluation, drug use, and family illness is just as essential as a careful neurologic examination when evaluating a patient with parkinsonism. Although there is no definitive diagnostic test for PD at this time, tests to rule out other causes should be considered and then treatment started.

Alzheimer Disease↗
PubMed1999

How to help patients with restless legs syndrome. Discerning the indescribable and relaxing the restless.

Restless legs syndrome is a common, potentially disabling condition that affects about 10% to 15% of the general population and yet is often unrecognized and misdiagnosed. It is mainly diagnosed clinically and only rarely requires polysomnography. The condition is usually primary and treatable. First, however, secondary causes should be sought, especially iron deficiency and peripheral neuropathy, because when the source is an accompanying factor or condition, the syndrome may be curable. The most effective drugs are dopaminergic agents, clonazepam, opioids, gabapentin, and clonidine. Additional agents are available that may be beneficial as add-on or alternative therapy.

Anti-Anxiety Agents↗
PubMed1998

Randomized, placebo-controlled study of tolcapone in patients with fluctuating Parkinson disease treated with levodopa-carbidopa. Tolcapone Fluctuator Study Group III.

OBJECTIVE: To assess the efficacy and tolerability of the catechol-O-methyltransferase inhibitor tolcapone in reducing "off/on" fluctuations in levodopa-treated parkinsonian patients. DESIGN: A randomized, double-blind, placebo-controlled, parallel-group study. SETTING: Fifteen Parkinson disease clinics. PATIENTS: Two hundred fifteen referred outpatients with Parkinson disease who showed predictable end-of-dose motor fluctuations that were not controlled by a stable levodopa-carbidopa (Sinemet) regimen of at least 4 weeks' duration. INTERVENTIONS: In addition to their usual levodopa-carbidopa regimen, patients received placebo or tolcapone, 100 or 200 mg, 3 times daily orally for 6 weeks. PRIMARY OUTCOME MEASURE: Change in daily off/on time. RESULTS: Tolcapone, 100 and 200 mg 3 times daily, reduced off time by 2.0 and 2.5 hours per day, respectively, and increased on time by 2.1 and 2.3 hours per day, respectively (P<.001 vs placebo). Investigators' global measures of disease severity indicated that significantly more tolcapone-treated patients had reduced wearing off and symptom severity (P<.001 vs placebo). No significant change in quality-of-life measures occurred. Clinical improvements occurred despite a reduction in total daily levodopa dose of 185.5 mg (23%) in the tolcapone, 100 mg 3 times daily, group and 251.5 mg (29%) in the 200 mg 3 times daily group. Principal adverse events (mainly dyskinesia and nausea) were levodopa related, were not treatment limiting, and were seldom reported as reasons for withdrawal. The frequency of withdrawals because of adverse events was similar in all groups (3% to 7%). CONCLUSIONS: Tolcapone was well tolerated and substantially increased on time and reduced off time in patients with fluctuating Parkinson disease. Additionally, levodopa requirements were significantly decreased.

Aged↗
PubMed1998

Ropinirole for the treatment of early Parkinson disease: a 12-month experience. Ropinirole Study Group.

OBJECTIVE: To evaluate ropinirole hydrochloride as dopaminergic monotherapy in patients with early Parkinson disease. DESIGN: A 6-month extension of a double-blind, placebo-controlled study. SETTING: Ambulatory care at 22 different sites in the United States. PATIENTS: Patients who successfully completed the initial 6-month study could enter the 6-month extension study (ropinirole, n = 70; placebo, n = 77). INTERVENTION: Use of ropinirole or placebo therapy. MAIN OUTCOME MEASURES: The efficacy variables were the number of patients who successfully completed the 12-month study and did not require supplemental levodopa, the number of patients requiring supplemental levodopa, and the proportion of patients having an insufficient therapeutic response. RESULTS: Significantly fewer ropinirole-treated patients met criteria for insufficient therapeutic response (23 [19.8%] of 116) or required the initiation of levodopa therapy (22 [19%] of 116) compared with placebo-treated patients (60 [48%] of 125 patients for insufficient therapeutic response; 57 [45.6%] of 125 patients for additional levodopa). Significantly more ropinirole-treated patients (51 [44.0%] of 116) successfully completed the 12-month study and did not require supplemental levodopa compared with placebo-treated patients (28 [22.4%] of 125). The incidence of adverse experiences and patient withdrawals was low. CONCLUSION: Ropinirole was effective and well tolerated as monotherapy for 12 months in patients with early Parkinson disease.

Adult↗
PubMed1998

Cortical myoclonus in levodopa-responsive parkinsonism.

We observed myoclonic movements of the fingers and wrists in two patients with a levodopa-responsive parkinsonian syndrome most consistent with Parkinson's disease. These patients were studied with electrophysiological techniques. Brief (<50 ms) myoclonic electromyographic discharges showed a time-locked relationship to a focal premovement electroencephalographic potential. Somatosensory-evoked potentials were not enlarged and long-latency reflexes were not grossly exaggerated. This pattern of electrophysiological findings can be distinguished from those previously found in other parkinsonian syndromes. These results provide evidence for a cortical origin of the myoclonus seen in these patients.

Aged↗
PubMed1998

Olfactory function in restless legs syndrome.

Restless leg syndrome (RLS) is usually idiopathic but may occur in patients with Parkinson's disease (PD). Both respond to dopaminergic medications. Whether these disorders share a common pathophysiology is unclear. Because PD is associated with a loss of olfactory function, we compared the olfactory function of patients with RLS with control and PD patients. Using the University of Pennsylvania Smell Identification Test (UPSIT), olfactory function was found to be normal in patients with idiopathic RLS and significantly reduced in patients with PD. This suggests that the pathophysiology of RLS differs from PD, and that RLS likely is not a "forme fruste" or a preclinical sign of PD.

Aged↗
PubMed1998

Erdheim-Chester disease with extensive intraaxial brain stem lesions presenting as a progressive cerebellar syndrome.

We report a rare case of Erdheim-Chester disease (ECD) presenting as a progressive cerebellar syndrome and diabetes insipidus. On magnetic resonance imaging, a 7-mm extraaxial, enhancing mass was seen enveloping the right vertebral artery and was confirmed at autopsy to represent an adventitial xanthoma with lipid-laden, foamy histiocytes. The cerebellar syndrome most likely resulted from extensive histiocytic infiltration of the pons, particularly the basis pontis and middle cerebellar peduncles.

Aged↗
PubMed1998

Effective treatment of orthostatic tremor with gabapentin.

We report seven patients with orthostatic tremor (OT) who were successfully treated with the anticonvulsant gabapentin. Five of the patients had been previously tried on clonazepam, the most commonly used drug for OT, four without any benefit. The degree of improvement perceived by the patients with gabapentin varied from 60-80% (mean 73%). The effective dose of gabapentin ranged from 300-1800 mg/day (mean 1030 mg/day). The side effects were generally mild, transient, and dose-related. Duration of response has so far ranged from 2-22 months (mean 11 months) with all patients still currently benefiting from gabapentin. We conclude that gabapentin may be an effective treatment for OT. Further trials are indicated.

Acetates↗
PubMed1998

Hemifacial spasm and other craniofacial movement disorders.

Craniofacial dyskinesias encompass a variety of abnormal spontaneous craniofacial movements that often appear similar in morphology but are, in fact, of varied cause and nature. Although hemifacial spasm and blepharospasm are the two most common abnormal craniofacial movements, the clinician should be cognizant of other dyskinesias, particularly craniofacial dystonias, tremor, tic, chorea, and stereotypies. Most craniofacial dyskinesias respond favorably to injections of botulinum toxin type A or oral medications. Surgical treatment may be beneficial for refractory cases.

Face↗
PubMed1998

Stereotactic pallidotomy for the treatment of Parkinson's disease. Efficacy and adverse effects at 6 months in 26 patients.

We evaluated the safety and efficacy of microelectrode-guided stereotactic pallidotomy in patients with advanced Parkinson's disease (PD). Using diagnostic criteria and evaluations outlined in the Core Assessment Programme in Transplantation (CAPIT) protocol, we studied unilateral pallidotomy in 26 patients with advanced idiophatic PD, motor fluctuations, and peak dose dyskinesias. All underwent unilateral stereotactic pallidotomy. Assessments conducted in the "practically defined off" and "best on" states at baseline and at 1 and 6 months postoperatively included Unified Parkinson's Disease Rating Scale (UPDRS) parts II, III, and IV and timed motor testing as outlined in CAPIT. Motor UPDRS in the "off" state improved at 1 and 6 months after surgery (p = 0.002, p = 0.008) Likewise, the sum of individual "off" contralateral motor UPDRS items improved (p = 0.0002, p = 0.0005). The duration (p = 0.0001 at 1 and p = 0.001 at 6 months) and severity (p = 0.003 at 1 and p = 0.0005 at 6 months) of dyskinesia improved, but other aspects of the "on" function were unchanged. Serious adverse effects occurred in eight patients and included one fatal deep and three nonfatal frontal lobe hemorrhages with resultant language or behavioral deficits. Nonhemorrhagic complications included one hemiparesis and three frontal lobe syndromes. Pallidotomy improves PD motor disability in the "off" state. Peak dose dyskinesias are reduced, although other aspects of "on" motor function are unchanged. Although morbidity may limit its use, pallidotomy is effective in targeting particular symptoms such as unremitting dyskinesia and severe "off" motor disability in advanced PD.

Activities of Daily Living↗
PubMed1998

COMT inhibition: a new treatment strategy for Parkinson's disease.

During the initial stages of Parkinson's disease, treatment with levodopa plus a decarboxylase inhibitor (carbidopa or benserazide) provides adequate control of symptoms. However, as the disease progresses, the clinical response to treatment often begins to fluctuate, becoming increasingly correlated with fluctuations in plasma concentrations of levodopa-the "wearing-off" phenomenon. Many strategies have attempted, with various degrees of success, to increase the availability of levodopa and its active metabolites, thus reducing these fluctuations in response. This review focuses on the role of the new catechol O-methyltransferase (COMT) inhibitors tolcapone and entacapone as adjuncts to levodopa therapy. These agents act effectively and safely to increase the amount of levodopa that is available to enter the brain by extending the half-life of levodopa, resulting in more stable levels in the plasma and prolonging "on" time.

Antiparkinson Agents↗
PubMed1997

Dystonia secondary to electrical injury: surface electromyographic evaluation and implications for the organicity of the condition.

We describe a patient who developed right arm dystonia following an electrical injury. The patient's arm remained adducted, and flexed at the elbow and wrist, with all movement resulting in pain and tremor. Surface electromyographic evaluation revealed constant tonic activity of multiple upper and lower arm muscles at rest, that was not distractible. Voluntary and passive movement of the elbow or wrist resulted in high amplitude EMG activity, with motor grouping at 11 Hz at the elbow and 8 Hz at the wrist. Although a diagnosis of psychogenic dystonia was entertained, the stereotyped nature of the movement disorder and lack of variability on clinical and surface EMG evaluation support an organic disorder that was temporally-related to an electrical injury.

Adult↗
PubMed1997

Parkinsonism reduces coordination of fingers, wrist, and arm in fine motor control.

This experiment investigates movement coordination in Parkinson's disease (PD) subjects. Seventeen PD patients and 12 elderly control subjects performed several handwriting-like tasks on a digitizing writing tablet resting on top of a table in front of the subject. The writing patterns, in increasing order of coordination complexity, were repetitive back-and-forth movements in various orientations, circles and loops in clockwise and counterclockwise directions, and a complex writing pattern. The patterns were analyzed in terms of jerk normalized for duration and size per stroke. In the PD subjects, back-and-forth strokes, involving coordination of fingers and wrist, showed larger normalized jerk than strokes performed using either the wrist or the fingers alone. In the PD patients, wrist flexion (plus radial deviation) showed greater normalized jerk in comparison to wrist extension (plus ulnar deviation). The elderly control subjects showed no such effects as a function of coordination complexity. For both PD and elderly control subjects, looping patterns consisting of circles with a left-to-right forearm movement, did not show a systematic increase of normalized jerk. The same handwriting patterns were then simulated using a biologically inspired neural network model of the basal ganglia thalamocortical relations for a control and a mild PD subject. The network simulation was consistent with the observed experimental results, providing additional support that a reduced capability to coordinate wrist and finger movements may be caused by suboptimal functioning of the basal ganglia in PD. The results suggest that in PD patients fine motor control problems may be caused by a reduced capability to coordinate the fingers and wrist and by reduced control of wrist flexion.

Adult↗