Atypical cases of acute headaches of cerebrovascular origin

Acute headaches can be an important signal of a cerebrovascular event. In some cases, as illustrated in this article, such headaches may have the same characteristics as migraine or be the main feature of unusual types of cerebrovascular disease. Headache should not be underestimated since misdiagnosis of cerebrovascular disease can lead to serious consequences.     

Periodic dystonia

20 authors' personal observations of periodic dystonia, a rare neurological syndrome, are presented. Main clinical manifestations are discussed in detail: the age of the disease debut, provoking factors, duration and frequency of the fits, the motor pattern, daily distribution of paroxysms. The comparison with analogous literary data is given too.     

Acute dystonia

Acute dystonia is a side effect of antipsychotic medication; it nearly always develops a few weeks after the start of a dopamine-blocking agent or substantial increase of the dosage. Acute dystonia is characterized as a syndrome of sustained muscle contractions, frequently causing twisting and repetitive movements or abnormal postures. The risk of acute dystonia depends greatly on the presence of risk factors: early age, male sex, use of cocaine, a history of acute dystonia, and use of a highly potent antipsychotic agent in a normal dosage. The mechanism underlying acute dystonia is unknown: both increase and decrease of the striatal dopamine transmission have been put forward as possible causes. Acute dystonia may also be caused by dopamine-blocking agents that are used not as antipsychotic medication but, for instance, as anti-emetics. Anticholinergic agents are extremely efficacious in treatment as well as prevention of acute dystonia. Prophylaxis of acute dystonia is indicated for patients belonging to the risk groups.     

The common marmoset (Callithrix jacchus) as a model for neuroleptic-induced acute dystonia

To examine whether acute dystonia is induced by neuroleptic treatment, common marmosets were treated with haloperidol orally twice a week over 25 weeks until dystonic behavior was elicited. Movement disorders such as acute dystonia were observed 6 weeks after the initial treatment, and had appeared in all treated animals by 25 weeks. Once these movement disorders were induced, they consistently reappeared after further treatment with haloperidol, and once haloperidol dosing was discontinued, the episodes vanished. Then, various neuroleptic drugs (bromperidol, chlorpromazine, risperidone thioridazine, sulpiride, tiapride, and clozapine) or a nonneuroleptic drug (diazepam) were administered orally instead of haloperidol in the above animals. All the neuroleptic drugs except for clozapine elicited similar abnormal behavior, while diazepam failed to induce any dystonia. An anticholinergic drug, trihexyphenidyl, which is known to reduce acute dystonia in patients, was also given orally to the above haloperidol-sensitized animals, followed by further treatment with haloperidol 30 min later. This clearly suppressed the induction of dystonia by haloperidol. The similarity between these findings for haloperidol-pretreated common marmosets and clinical findings suggests that the present model is useful for predicting the potential of antipsychotics to induce acute dystonia in humans.     

Posttraumatic cervical dystonia

Posttraumatic cervical dystonia has been described as a distinct syndrome with some similarities to idiopathic nontraumatic cervical dystonia (torticollis). We describe five patients in whom cervical dystonia developed immediately after relatively mild trauma to the neck. Four of the five patients had persistent contractions of all cervical muscles including the trapezius muscles, which almost completely prevented motion of the neck and resulted in muscle hypertrophy. The condition persisted unabated in all patients for the entire period of follow-up (duration, 1 1/2 to 3 years). Pharmaceutical interventions, which had been used previously for idiopathic nontraumatic cervical dystonia, failed to benefit these patients. Two patients who received injections of botulinum toxin had no more than mild benefit. Selective denervation was inapplicable because of the widespread involvement of all cervical muscles in all but one patient. Physical therapy was essentially ineffective. Because of the unusual features and possible medicolegal setting, clinicians may tend to diagnose this condition as a psychogenic disorder or litigation-oriented behavior. The clinical picture, however, is consistent with an organic dystonia that may render the patient functionally and occupationally disabled.     

Pallidotomy for generalized dystonia

OBJECTIVE: The objective of this study was to determine the predictors of future ambulatory blood pressure in normotensive youths with family histories of essential hypertension. STUDY DESIGN: Eighty-eight healthy youths (mean age 10.9 +/- 2.5 years; 52 blacks, 36 whites; 45 boys) were studied. During an initial visit anthropometric variables and hemodynamics were measured at rest and before, during, and after three laboratory stressors: postural change, forehead cold, and video game challenge. The subjects' ambulatory blood pressure was monitored for 24 hours as part of a follow-up evaluation an average of 2.5 years later. RESULTS: Anthropometric and demographic variables and measures of reactivity to laboratory stressors were related to future daytime and nighttime ambulatory blood pressure. CONCLUSION: These findings provide important information on the predictors of ambulatory blood pressure and underscore the importance of resting blood pressure and adiposity. These results support the guidelines of the Second Task Force, which recommend the measurement of blood pressure and adiposity in the context of ongoing health care.     

MPTP induces dystonia and parkinsonism. Clues to the pathophysiology of dystonia

The pathophysiology of dystonia is unclear, but several clues implicate striatal dopamine dysfunction. In contrast, the causal relationship between striatal dopamine deficiency and parkinsonism is well defined. We now suggest that parkinsonism or dystonia may occur following striatal dopamine deficiency. Baboons treated with intracarotid 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) developed transient hemidystonia prior to hemiparkinsonism. The day after MPTP treatment, most animals had spontaneous ipsilateral turning. Within a few days, all developed contralateral hemidystonia, with the arm and leg extended and externally rotated. This transient dystonia preceded hemiparkinsonism with flexed posture, bradykinesia, and postural tremor that persisted for up to 1.5 years. Dystonia corresponded temporally with a decreased striatal dopamine content and a transient decrease in D2-like receptor number. The time course of dystonia and parkinsonism is analogous to lower limb dystonia as the first, frequently transient, symptom of Parkinson's disease in humans. The association of striatal dopamine deficiency with dystonia and parkinsonism implies that other factors influence clinical manifestations.     

Atypical hand infections

The diagnosis and treatment of an atypical hand infection present a distinctive challenge for the hand surgeon. Infections caused by these uncommon organisms occur more often in immunocompromised patients. This article emphasizes the salient features of mycobacterial, nocardial, and fungal infections of the hand. With an accurate and timely diagnosis, appropriate surgical and pharmacologic treatment may eradicate these unusual infections.     

Atypical Kaposi's disease

To demonstrate that Kaposi's sarcoma may initially take several different courses, a case is presented and the literature reviewed.     

Atypical Parkinson syndrome

Various clinico-pathological studies have shown that appr. 20% of patients with a clinical diagnosis of idiopathic Parkinson's disease (IPD) may have neuropathological evidence of alternative causes of parkinsonism. Most of these misdiagnosed "IPD" patients meet clinical criteria for either multiple system atrophy (MSA), or progressive supranuclear palsy (PSP), or corticobasal degeneration (CBD). A careful history and physical examination, as well as follow-ups and selected investigations are essential for an accurate clinical diagnosis of these atypical parkinsonian syndromes. The following paper therefore provides a review of clinical features and diagnostic findings in MSA, PSP and CBD, in order to facilitate recognition of these patients.     

Atypical chest pain

The initial approach to patients with a chief complaint of chest pain is to rule out myocardial ischemia. There are, however, other life-threatening causes of chest pain, including pulmonary emobilism and aortic dissection among many others. This article reviews several of these disease processes.     

Comparison of treatment of tardive dystonia and idiopathic cervical dystonia with botulinum toxin type A

To compare clinical parameters of patients treated with botulinum toxin type A (BTX) for treatment of idiopathic cervical dystonia (ICD) and for tardive cervical dystonia (TCD), we studied 156 patients (149 with ICD and 7 with TCD) who were treated with serial injections of BTX over 5 years. We hypothesized that patients with TCD and ICD would demonstrate similar improvement in severity scores after treatment with BTX. The diagnosis, dates, dosages, and frequency of BTX injected and severity assessments were recorded into a computerized database. Nonparametric Wilcoxon rank sum and signed-rank tests were used to assess statistical significance. The change in severity scores between the first treatment and last treatment in both groups was not statistically significant (p = 0.4859), indicating similar improvement. The difference in BTX doses was significant (p = 0.0045). ICD patients (n = 149) received an average of 219.8 +/- 63.5 units and those with TCD (n = 7) were treated with an average dose of 287.4 +/- 60.3 units. The average number of days between treatments for individuals with ICD was 142.9 +/- 85.8, similar to that for persons with TCD (144.7 +/- 64.5) (p = 0.6075). Our analysis provides preliminary evidence that the improvement from the administration of BTX for patients with ICD and TCD is similar.     

Focal dystonia and repetitive motion disorders

It commonly is observed that focal hand dystonias, such as writer's cramp or musician's cramp, are associated with repetitive movements, although definitive proof of a causal relationship is lacking. These focal dystonias are often task specific, with involuntary muscle contractions occurring only when patients perform specific acts such as writing or playing a musical instrument. Physiologic studies show deficiencies in spinal reciprocal inhibition and abnormalities of central sensory processing and motor output that may be related to reduced cortical inhibition. Recent studies in primates support the notion that repetitive motions can induce plasticity changes in the sensory cortex leading to degradation of topographic representations of the hand, and raise the possibility that sensory training may be beneficial. Current treatment options for focal dystonia include botulinum toxin injections, anticholinergics, baclofen, benzodiazepines, and occupational therapy.     

Putamen volume in idiopathic focal dystonia

PURPOSE: To determine the accuracy of magnetic resonance (MR) imaging in evaluating the invasive cervical carcinoma response to concurrent chemotherapy and radiation therapy. MATERIALS AND METHODS: MR imaging was performed before and after concurrent chemotherapy and radiation therapy in 18 patients with locally invasive cervical carcinoma. Surgery followed neoadjuvant therapy in all patients. The presence of a lesion, signal intensity, zonal anatomy integrity, vaginal and parametrial invasion, and lymph node enlargement was determined. Posttreatment MR and histopathologic findings were correlated. RESULTS: Fourteen patients had histopathologic confirmation of MR findings: Twelve had true-negative and two had true-positive findings. (Two had microscopic neoplastic foci beyond the spatial resolution of MR images; these foci do not change surgical treatment planning and probably do not influence prognosis. Therefore, these two patients were considered to have complete response). Four patients had false-positive findings; the hyperintense lesion on posttreatment MR images was due to a tunnel cluster pattern (focal hyperplasia of the endocervical glands with inflammation) in three patients and necrosis in one patient, without any evidence of neoplastic tissue. Thirty-three of 36 parametrial halves and 67 of 72 vaginal fornices were correctly interpreted on posttreatment images. Involvement of three parametrial halves and five fornices was overestimated at MR, because edema or inflammation was not distinguishable from tumor. CONCLUSION: MR imaging is 78% accurate in evaluation of tumor response; in 22% of patients, however, benign conditions were not distinguishable from tumor.     

Early onset dystonia decreasing with development. Case report of two children with familial myoclonic dystonia

Two related girls had the onset of unilateral leg dystonia in the neonatal period and at 13 months, respectively. The dystonic signs subsided with motor development and resolved completely in one of the girls by the age of 5 years. There was no response to L-dopa. From 2-3 years of age segmental myoclonus with a shoulder girdle distribution appeared. Family investigation results were compatible with autosomal dominant myoclonic dystonia responsive to alcohol. The onset and resolution of dystonia have not been described previously. This disorder is genetically separate from torsion dystonia. No linkage has been found to the dopamine beta-hydroxylase gene locus. Genetically determined disorders of neurotransmission may add to our knowledge of the normal development of motor control and thus merit further study.