Deanol Acetamidobenzoate Treatment in Choreiform Movement Disorders
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Abstract:
• Deanol acetamidobenzoate was administered in double-blind, crossover fashion with placebo to five patients with tardive dyskinesia, three patients with Huntington's chorea, and one patient with posthemiplegic chorea. No significant effect on dyskinesia was observed. Preliminary administration of physostigmine salicylate to patients with tardive dyskinesia had a variable effect, while benztropine mesylate produced no change. Since the status of deanol as an effective precursor of acetylcholine is uncertain, further trials with putative cholinergic agents remain warranted in choreiform syndromes.Keywords:
Tardive dyskinesia
Choreiform movement
Physostigmine
Benztropine
Crossover study
Movement Disorders
• Deanol acetamidobenzoate was administered in double-blind, crossover fashion with placebo to five patients with tardive dyskinesia, three patients with Huntington's chorea, and one patient with posthemiplegic chorea. No significant effect on dyskinesia was observed. Preliminary administration of physostigmine salicylate to patients with tardive dyskinesia had a variable effect, while benztropine mesylate produced no change. Since the status of deanol as an effective precursor of acetylcholine is uncertain, further trials with putative cholinergic agents remain warranted in choreiform syndromes.
Tardive dyskinesia
Choreiform movement
Physostigmine
Benztropine
Crossover study
Movement Disorders
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Eleven patients with Huntington chorea and nine with delayed dyskinesia were participants in a randomized double blind cross-over study of dogmatil (as the single antidopaminergic treatment) versus placebo. Although we recorded no functional improvement in Huntington chorea patients, dogmatil reduced the frequency of movements and the overall dyskinesia score in both conditions. From a pharmacologic standpoint, dogmatil differs on several accounts from conventional neuroleptics and there is no conclusive evidence that it may produce delayed dyskinesia. Among currently available treatments, dogmatil emerges as the drug of choice for the treatment of delayed dyskinesia.
Tardive dyskinesia
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The acute onset of a unilateral choreiform movement dis order in a child with a recently documented febrile illness or streptococcal pharyngitis is suggestive of Sydenham chorea. The MR manifestations of two clinically corroborated cases of Sydenham chorea are summarized. Increased signal inten sity in the corpus striatum contralateral to the patients' symp tomatic side was observed on T2-weighted images. Focal enhancement in the involved head of the caudate was iden tified after IV administration of gadopentetate dimeglumine. The MR findings correlated with anatomic regions , which have been described to be susceptible to cross reaction with lgG antibodies that are formed in association with streptococcal infection.
Choreiform movement
Petit mal
Movement Disorders
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Choreiform movement
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Chorea is caused by a number of conditions, including genetic, metabolic derangements, infections, drugs, toxins, tumors, and disorders of the immune and inflammatory system of the body. Huntington's disease (HD) is the most common genetic cause of chorea. Systemic lupus erythematosus (SLE) is an autoimmune condition. Common symptoms include oral ulcers, joint pain, malar or discoid rashes, photosensitivity, and blood dyscrasias. It can involve the heart, lungs, kidneys, and brain. SLE can cause neuropsychiatric manifestations like psychosis, seizures, headache, confusion, and stroke. Chorea is a known symptom of SLE. HD is now recognized to involve more than one system and is associated with a number of comorbid conditions. We report the first case of hereditary choreiform disorder associated with and aggravated by SLE. This is also the first case report of probable Huntington disease from Balochistan, Pakistan. We report a 19-year-old girl with choreiform disorder and a family history of chorea. Choreiform disorder was present in her paternal grandmother and uncles. She presented with fever, cough, and aggravation of choreiform movements of upper and lower limbs for 10 days. She also complained of pain in the small joints of her hands and feet, oral ulcers, hair loss, and aggravation of choreiform movements for two and half months. Probable differential diagnoses of HD, Wilson's disease, and other types of hereditary chorea, aggravated by infections, SLE, or Covid-19, were made. Her initial lab results revealed pancytopenia, increased D-dimers and serum ferritin, positive antinuclear antibodies (ANA), and anti-double-stranded DNA (anti-dsDNA). Her C3 and C4 complement factors were low. The rest of the lab test results, including polymerase chain reaction (PCR) coronavirus disease (COVID-19), blood culture, and malaria, were negative. Thus, a diagnosis of hereditary chorea associated with and aggravated by SLE was made. Hereditary choreiform disorders can be associated with and aggravated by autoimmune conditions like SLE. Thus, it is recommended to be vigilant and have a low threshold for diagnosing co-existing autoimmune conditions like SLE in patients with hereditary choreiform disorder.
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Clinical features suggesting a diagnosis of Huntington's chorea in several siblings in a Black Rhodesian family are described. A progressive and eventually fatal mental deterioration accompanied by gradually worsening choreiform movements has until the present time occurred in only a single generation of this family, which precludes a definitive diagnosis based on the triad of family history, chorea and dementia. It is suggested that Huntington's chorea is the most likely diagnosis.
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We report the case of 4-year-old male with sinus venous thrombosis leading to bilateral thalamic and basal ganglia strokes presenting as generalized choreiform movements. Acute-onset chorea in the pediatric population is most commonly associated with Sydenham chorea, which is a manifestation of acute rheumatic fever. Chorea is a much less commonly noted sign of stroke, and when it occurs, it typically presents as hemichorea. Given the unlikely presentation, rapid and appropriate imaging was the key to diagnosis.
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