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Persistent hand spasm: movement disorder or seizure?

Dystonia is a movement disorder characterized by involuntary, sustained or repetitive, patterned muscle contractions or spasms, frequently causing squeezing and twisting movements or abnormal postures. Dystonic posturing could be one of the accompanying features of epilepsy. We report an elderly lady with intractable focal seizure who presented with more than a year of persistent hand spasms. The clinical importance of differentiating epilepsy from movement disorders is discussed.

Aged↗

Low-frequency repetitive TMS of premotor cortex can reduce painful axial spasms in generalized secondary dystonia: a pilot study of three patients.

Dystonia is associated with excessive corticospinal motor output. Motor cortex excitability may be reduced by low-frequency repetitive transcranial magnetic stimulation (rTMS) of premotor cortical areas. We report the effects of 1 Hz rTMS applied at 90% of resting motor threshold over the left premotor cortex in an open pilot study of three patients with severe, generalized, secondary dystonia including painful spasms in the proximal and axial musculature. A 20-min session of premotor rTMS was daily performed during 5 consecutive days. The series of rTMS sessions dramatically reduced the painful spasms, for 3-8 days after the last session, without any other significant beneficial effects. However, a slight reduction of the Movement score of the Burke, Fahn and Marsden rating scale was observed for two patients, and of the Disability score for the third one. Low-frequency rTMS of the premotor cortex may improve some specific motor symptoms in severe, generalized dystonia. These results should prompt confirmation in a larger placebo-controlled study.

Adult↗

Operative prevention and management of voice-limiting pharyngoesophageal spasm.

Voice-limiting pharyngoesophageal spasm presents a challenge to the surgeon who is trying to optimize functional voice outcomes in the laryngectomy patient. Modified closure techniques, adjunctive myotomy, and pharyngeal plexus neurectomy have the ability to improve or prevent pharyngoesophageal spasm. Attempts to maximize vocal outcome should be considered at the original time of laryngectomy and tracheoesophageal voice restoration because salvage treatment can be challenging.

Botulinum Toxins, Type A↗

Coronary artery spasm as the cause of myocardial infarction during coronary arteriogarphy.

Catheter-induced coronary artery spasm has been observed frequently. It is usually transient, reacts to the administration of nitroglycerin, and its distribution is generally confined to an area in proximity ot the intubated catheter. A 43-year-old woman with recurrent chest pain was found to have a rather long segment of tight proximal obstruction of the right coronary artery and experienced a myocardial infarction during coronary catheterization. Because of recurrent attacks of severe chest pain, coronary artery bypass surgery was performed which failed to result in significant improvement of her symptoms. Two repeat coronary cineangiograms seven weeks and three years after surgery revealed the proximal right coronary artery to be free of stenotic lesions or of luminal irregularities. After considering possible mechanisms of myocardial necrosis in the presence of normal coronary arteries it is concluded that myocardial necrosis can result from catheter-induced coronary artery spasm in spite of administration of nitroglycerin.

Adult↗

Coronary spasm with ventricular fibrillation during thyrotoxicosis: response to attaining euthyroid state.

Although myocardial ischemia may occur in thyrotoxic patients with normal coronary arteries, the mechanism remains unclear. This report describes a woman with hyperthyroidism who had ventricular fibrillation during an apisode of myocardial ischemia. The event was documented with continuous ambulatory electrocardiography. Subsequent angiography revealed normal coronary anatomy with spasm of the right coronary artery that disappeared after ingestion of one sublingual nitroglycerin tablet. The angina, electrocardiographic evidence of myocardial ischemia, ventricular arrhythmias and the patient's need for nitroglycerin were eliminated after she became euthyroid. These findings suggest that coronary spasm may be associated with myocardial ischemia and arrhythmias in a hyperthyroid patient.

Coronary Angiography↗

Spasm of the near reflex associated with organic disease.

Spasm of the near reflex is characterized by transient attacks of convergence, accommodation, and miosis. It is usually observed in young individuals and considered functional. We studied seven patients with spasm of the near reflex who had associated neurologic disorders or head trauma. Two patients had posterior fossa abnormalities (cerebellar tumor, Arnold-Chiari malformation), two patients had pituitary tumors, one patient had a vestibulopathy, and two patients had a history of antecedent head trauma.

Accommodation, Ocular↗

Facial nerve injury and hemifacial spasm.

We studied evidence of facial nerve damage in patients with hemifacial spasm. Three types of evidence of nerve damage were analyzed: objectively measured weakness in eyelid protractor strength, clinically evident weakness of muscles innervated by the seventh nerve, and clinically evident aberrant seventh nerve regeneration. Of the 60 patients in the study, 54 (90%) had at least one of these features of seventh nerve damage. Objectively measured eyelid protractor weakness was noted in 27 of 58 patients (47%) who were tested. Clinically apparent weakness of at least one of four facial muscle groups was noted in 42 of 60 patients (70%). Aberrant seventh nerve regeneration was documented in 25 of 60 patients (42%). These findings indicate that facial nerve damage is common in patients with hemifacial spasm.

Adult↗

Nitroglycerin is preferable to diltiazem for prevention of coronary bypass conduit spasm.

BACKGROUND: Diltiazem is widely used to prevent radial artery spasm after coronary bypass grafting (CABG). However, recent in vitro and in vivo studies have shown that nitroglycerin is a superior conduit vasodilator compared to diltiazem. A clinical comparison of these agents in patients undergoing CABG has not been previously performed. METHODS: One hundred sixty-one consecutive patients undergoing isolated CABG with the radial artery were prospectively randomized to 24-hour intravenous infusion of nitroglycerin or diltiazem followed by 6-month treatment with a daily dose of isosorbide mononitrate (n = 84) or diltiazem CD (n = 77). Analyses were performed on "intention-to-treat" basis. RESULTS: Crossovers because of low cardiac output, complete heart block, or sinus bradycardia occurred in 5 patients in the diltiazem group and none in the nitroglycerin group (p = 0.05). Operative mortality (nitroglycerin, 1.2% versus diltiazem, 1.3%), major morbidity (14% versus 16%), perioperative myocardial infarction (1.2% versus 0%), peak serum creatinine phosphokinase MB fraction levels (27 versus 21 U), intensive care unit stay (34+/-19 versus 38+/-30 hours) and total hospital length of stay (4.7+/-1.4 versus 4.7+/-1.3 days) were similar (p = not significant for all). Cardiac pacing was required more often in the diltiazem group (28% versus 13%, p = 0.01). Follow-up longer than 2 months was available in 145 patients (90%). Follow-up mortality (nitroglycerin, 1.2%; diltiazem, 1.3%), myocardial infarction (6%, versus 5%), and reintervention (8% versus 6%) rates and average angina class (1.3+/-0.7 versus 1.1+/-0.4) were similar (p = not significant for all). Thallium stress test obtained in 117 patients showed abnormal perfusion in the radial artery territory in only 4 patients (3%), 2 in each group (p = not significant). Treatment with diltiazem was more costly ($16,340 versus $1,096). CONCLUSIONS: Nitroglycerin is preferable to diltiazem for prevention of conduit spasm. Nitroglycerin is safe, effective, better tolerated, and less costly than diltiazem, and therefore, should be the agent of choice.

Coronary Artery Bypass↗

Diffuse spasm of the esophagus.

During the past ten years 7 men and 15 women with diffuse esophageal spasm have been seen at the Duke University Medical Center. Dysphagia and severe substernal pain were the two characteristic symptoms. Eleven of the 22 patients were treated with a long esophageal myotomy. Two had a diverticulum of the lower esophagus excised in addition, while 6 had an associated sliding hiatal hernia repaired. Three patients in whom the diagnosis was made retrospectively all had an epiphrenic diverticulum excised without a myotomy; in 1 an esophageal leak occurred. These 3 patients still have mild symptoms of their diffuse esophageal spasm. The results of myotomy have been satisfactory. Although this operation does not correct the cause of the disorder, the improvement in symptoms makes it worthwhile in selected patients.

Adult↗

Infantile spasms as the initial symptom of biotinidase deficiency.

Two patients with biotinidase deficiency had diagnoses of infantile spasms made at 1 month of age. Biotinidase deficiency may be seen early in the neonatal period without the characteristic findings such as alopecia and seborrheic dermatitis. This diagnosis should be considered in patients with infantile spasms.

Amidohydrolases↗

Brain maturational aspects relevant to pathophysiology of infantile spasms.

Infantile spasms (IS) are so typically associated with West syndrome that the term IS, properly referred to as a seizure type, is currently used synonymously with this severe infantile epilepsy. This chapter reviews some clinical and experimental observations relevant to IS pathophysiology with particular regard to maturational aspects that may account for IS age-related expression. Neither the cortical nor the brain stem generator hypotheses account for all the clinical features of IS. It is suggested that IS are generated whenever a cortical discharge is able to influence some archaic circuit located putatively in the brain stem through either an excitatory or a disinhibitory mechanism. This may occur with the highest probability during a developmental window in which IS typically occur, when N-methyl-D-aspartate (NMDA) transmission is particularly facilitated due a reduced sensitivity of NMDA receptors to the Mg2+ block. Interestingly, in this age range (0-18 postnatal day in rat), flexion spasms can be elicited experimentally by NMDA administration. These data provide an important key to further investigating IS pathophysiology, thus allowing us to design more effective strategies aimed at counteracting this harmful infantile epilepsy.

Aging↗

Latanoprost in hemifacial spasm.

An empirical observation that the prostaglandin derivate latanoprost quited facial spasms in patients with glaucoma prompted us to study latanoprost in non-glaucomatous patients with hemifacial spasms [HFS]. This explorative trial followed an open-label, prospective treatment design. At short term, most patients showed no or only mild improvement and subsequently needed therapy with Botulinum toxin A. Although these results are not impressive, latanoprost could be considered as an interesting alternative in the treatment of patients with HFS and with concomitant glaucoma. A role for prostaglandins in the trigeminofacial reflex is hypothesised.

Aged↗

Clinical doxorubicin chemomyectomy. An experimental treatment for benign essential blepharospasm and hemifacial spasm.

Doxorubicin (DXR) was injected as a treatment for benign essential blepharospasm and hemifacial spasm. The other eyelids were treated concurrently with botulinum toxin (BT). No DXR-treated eyelid has maintained 0 strength (commonly achieved with BT). Two patients with benign essential blepharospasm and four patients with hemifacial spasm have achieved major improvement, sustained for more than 6 months. Eyelids have been swollen and inflamed for up to 3 months. No spontaneously irreversible complication has occurred. A single injection at the maximum safe dose (1 mg in the upper lid and 1.5 mg in the lower lid) has not proven sufficient to produce cure. Treatment of each lower eyelid of a muscular male with severe blepharospasm may require cumulative doses of up to 4.0 mg, delivered in three injection events separated by at least 2 months, with each injection no greater than 1.5 mg DXR per site. At the present time, there is no assurance that a permanent cure will result.

Aged↗

Temporal relationship modeling: DTP or DT immunizations and infantile spasms.

The time relationship between DTP immunization and infantile spasms (IS) onset was examined using three models--association, temporal shift, and no-effect--and the case/control data from the National Childhood Encephalopathy Study (NCES). Infantile spasms cases classified as being previously abnormal (e.g., tuberous sclerosis complex patients) showed a no-effect relationship, whereas those classified as previously normal suggested a fit to the temporal shift model, i.e. no increase in number of cases but a shortening of time to onset of seizure. No data fit the association model. Analyses for vaccine complications should examine for temporal changes (i.e. temporal shift) in addition to increased risks.

Child, Preschool↗

Spontaneous remission of spasms in West syndrome--implications of viral infection.

On a database of the Japanese medical literature between 1970 and 2000, there were 29 patients with West syndrome showing spontaneous remission. In 25/29 (86%) of the patients, spontaneous remission was preceded by viral infections, among which exanthema subitum predominated. In 16/29 (55%), the spasms recurred a median of 2 months later. However, some of the remaining infants attained lasting remission of the spasms. This link between viral infections and spontaneous remission suggests immuno-inflammatory processes underlie West syndrome in certain patients. Better understanding of such mechanisms may lead to a new therapeutic approach to West syndrome.

Autoimmune Diseases↗

Neuropathology of the brainstem in age-dependent epileptic encephalopathy--especially of cases with infantile spasms.

Twenty-two autopsy cases with developmental disabilities with history of infantile spasms were studied with special regard to brainstem lesions. Eleven out of 22 cases were considered to be of prenatal origin, ten cases were perinatal and one case had suffered from acute encephalopathy at six months of age. Some common pathological findings were noticed throughout the cases despite their various etiologies; small size of the brainstem tegmentum, spongy state in and around the central tegmental tract, localized periaqueductal glial scar. Control study on another autopsy series of 76 cases with developmental disabilities showed a close connection between these findings in the brainstem tegmentum and history of infantile spasms.

Adolescent↗

Neuropathological aspects of infantile spasms.

A review of the neuropathological findings in 50 personal autopsy cases and in the available literature (214 confirmed cases) of infantile spasms demonstrates that this type of early infantile epilepsy occurs in a wide range of cerebral lesions caused by various noxae during different stages of brain development. With regard to morphology and the presumed time of occurrence of the CNS lesions, four groups can be distinguished: (1) embryofetal lesions, including a) cerebral malformations or developmental disorders-agyria-pachygyria (lissencephaly), micrencephaly, micropolygyrias, (hemi)megalencephaly, agenesis of corpus callosum, tuberous sclerosis, heterotopias, cortical microdysplasias, b) metabolic disorders (leukodystrophies, neurolipidoses, spongy dystrophies, Leigh and Alpers diseases, aminoacidopathies); (2) perinatal and postnatal encephalopathies, e.g. polycystic brain, diffuse and lobar sclerosis, ulegyrias, white matter and basal ganglia scars, status marmoratus, hippocampal sclerosis, and cerebellar atrophy; (3) combined embryofetal (developmental) and perinatal or postnatal brain lesions, particularly association of microdysplasias with secondary anoxic or vascular changes; (4) acute vascular and inflammatory brain injuries; (5) cases without definite brain pathology. Evaluation of the available data indicates that embryo-fetal lesions alone or accompanied and/or superimposed by perinatal or postnatal lesions account for about 61% of the cases confirmed by autopsy, in which infantile spasms can be regarded as fetal epilepsies, while a smaller group is featured by perinatal or postnatal lesions occurring in early age, i.e. affecting the immature brain. Similar lesions are observed in cases showing transition of West syndrome to Lennox syndrome. Negative pathology findings in a small number of cases do not necessarily implicate negative pathobiology.(ABSTRACT TRUNCATED AT 250 WORDS)

Brain↗

Neuroradiological aspects of infantile spasms.

With the modern noninvasive brain imaging methods, cerebral lesions of different types and degrees can frequently be determined in infants with West syndrome. In CT examinations preceding the spasms and the ACTH therapy, "idiopathic" forms of infantile spasms were rare. The CT findings consistent with perinatal or postnatal encephalopathy were more frequent than those found with embryonic or fetal lesions alone. The fact that pathognomonic changes cannot be determined, may reflect the low specificity of CT diagnosis in infants with chronic CNS diseases. A slight and mostly transient enlargement of CSF spaces during ACTH therapy is a probable side-effect of the medication. In infants with persistent or progressive enlargement of the CSF spaces, however, this alteration appears to be mainly due to pre-existing brain damage. CT examinations of infants with West syndrome should be performed because prognostic information may be obtained and in a few cases operations to drain CSF may be necessary.

Humans↗