Manuel Fernandez Pardal

Flunarizine- and cinnarizine-induced extrapyramidal reactions.

Cinnarizine and flunarizine are selective calcium blockers that have been used to treat and prevent vertigo. We studied 15 patients who had extrapyrqmidal syndromes after taking these drugs. Eleven patients had parkinsonism, one with persistent akathisia as well; one had an orofacial tremor; one, acute akathisia alone; and one an acute dystonic reaction. All but one improved when the drug therapy was discontinued. Seven patients were also depressed during treatment. Cinnarizine and flunarizine must therefore be added to the list of potentially risky drugs known to induce extrapyramidal reactions and depression.

Bruxism secondary to chronic antidopaminergic drug exposure

Eight cases of diurnal bruxism (DB) secondary to long-term antidopaminergic drug exposure are reported. Five exhibited a grinding pattern, one a clenching form, and two a mixed type. An odontological etiology was absent throughout. EMG recordings disclosed two distinct patterns of muscle activity, one with brief rhythmic, forceful contractions and the other featuring sustained prolonged contractions. Surface EMG and EEG monitoring during a 24-h period confirmed the absence of bruxism during sleep. Several drug trials failed to provide relief. Our findings support DB as a focal tardive dystonia syndrome.

Holmes tremor: Clinical description, lesion localization, and treatment in a series of 29 cases.

Objective: To describe the clinical features, etiology, findings from neuroimaging, and treatment results in a series of 29 patients with Holmes tremor (HT). Methods: A retrospective study was performed based on review of medical records and videos of patients with HT diagnosis. Results: A total of 16 women and 13 men were included. The mean age at the moment of CNS insult was 33.9 ± 20.1 years (range 8–76 years). The most common causes were vascular (48.3%), ischemic, or hemorrhagic. Traumatic brain injury only represented 17.24%; other causes represented 34.5%. The median latency from lesion to tremor onset was 2 months (range 7 days–228 months). The most common symptoms/signs associated with HT were hemiparesis (62%), ataxia (51.7%), hypoesthesia (27.58%), dystonia (24.1%), cranial nerve involvement (24.1%), and dysarthria (24.1%). Other symptoms/signs were vertical gaze disorders (6.8%), bradykinesia/rigidity (6.8%), myoclonus (3.4%), and seizures (3.4%). Most of the patients had lesions involving more than one area. MRI showed lesions in thalamus or midbrain or cerebellum in 82.7% of the patients. Levodopa treatment was effective in 13 out of 24 treated patients (54.16%) and in 3 patients unilateral thalamotomy provided excellent results. Conclusions: The most common causes of HT in our series were vascular lesions. The most common lesion topography was mesencephalic, thalamic, or both. Treatment with levodopa and thalamic stereotactic lesional surgery seems to be effective.

Holmes tremor secondary to brainstem hemorrhage responsive to levodopa: report of 2 cases.

We report 2 patients who presented a brainstem hemorrhage and who, after 1 and 6 months, respectively, developed a 4-Hz postural and resting tremor consistent with Holmes tremor, which severely interfered with the activities of daily living. In both cases, levodopa dramatically improved the tremor. Pharmacological treatment of this condition is usually disappointing, and surgical procedures are commonly required for severe cases. Our patients, together with 13 others gleaned from the literature, suggest that in cases of Holmes tremor secondary to brainstem hemorrhage, levodopa can be a useful treatment, and it should be tested before considering invasive therapies.

Dopa-responsive dystonia masquerading as idiopathic kyphoscoliosis

A 19-year-old girl with a long-standing history of kyphoscoliosis misdiagnosed as idiopathic was offered corrective surgery on several occasions but fortunately refused, since neurological examination later found evidence of mild dystonic posturing in the neck and right leg. Symptoms worsened toward evening but improved with rest. Treatment with low doses of levodopa led to total remission within a month. Our case illustrates that dopa-responsive dystonia can manifest spinal curvature as the major symptom and warrants its inclusion in the differential diagnoses of idiopathic kyphoscoliosis.

Unusual phenotypic expression of the DYT1 mutation

Highly variable phenotype expression has long been recognized in DYT1 carrier patients. We report here an Ashkenazi-Jewish woman who carried a DYT1 mutation and developed a predominant unilateral myoclonic-dystonia (MD) displaying a fluctuating course. The present case is the second supporting the variability of DYT1 phenotype and further illustrates its ability to mimic the MD syndrome.

Paroxysmal Dystonia Responsive to Anticholinergic Drugs

A case of a mentally retarded patient with sporadic paroxysmal dystonia, unresponsive to anticonvulsant therapy, is described. He had a longstanding history of neuroleptic drug intake. Trihexyphenidyl in a total daily dosage of 20 mg totally suppressed the crises.

Beneficial effects of lisuride in Meige disease

We studied the therapeutic effects of apomorphine, bromocriptine, and lisuride on the dystonic spasms of two patients with Meige disease. Lisuride, a potent dopamine receptor agonist, and apomorphine had equal efficacy in lessening facial dyskinesia, while bromocriptine had no consistent effect. Lisuride can be an excellent drug in the treatment of this disabling disorder.

DOPA-RESPONSIVE DYSTONIC CAMPTOCORMIA

We read Dr. van Gerpen’s article in which he reports a patient with camptocormia remarkably responsive to levodopa treatment.1 The authors report that having the patient walk backwards improved the position of the patient’s spine, which was equated to a sensory trick. It was concluded that camptocormia was due to dystonia because of the presence of diurnal fluctuation and …

Playing harp, another unusual task-specific dystonia†

Herein we report a task‐specific dystonia in a 48‐year‐old woman, with an unusual association between a familial harp‐playing dystonia and essential tremor.