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PubMedDate not supplied

Painful tonic spasms caused by putaminal infarction.

Lacunar infarcts in the basal ganglia are known to cause various movement disorders, such as chorea, focal dystonia, and hemichorea-hemiballismus. We report here a case of putaminal lacunar infarction which presented with "painful tonic spasms" of the contralateral limbs. This consisted of paroxysmal brief, painful, flexor contractures of the upper, and occasionally the lower limb. These were not focal seizures but were controlled with carbamazepine, which has been used for the "painful tonic spasms" well-associated with multiple sclerosis. The putaminal infarct we describe is probably related to a lupus anticoagulant and systemic lupus erythematosus.

Adult↗
PubMed1984

Effect of nitroglycerin on regional myocardial blood flow following an experimental coronary spasm.

This study was designed to evaluate the effect of nitroglycerin (30 micrograms given as an i.v. bolus) on regional distribution of myocardial blood flow in conscious dogs, following an acute coronary occlusion similar to a coronary spasm. The left anterior descending (LAD) coronary artery was acutely occluded with a balloon cuff occluder. The distribution of blood flow between the endocardium and the epicardium of both the normal and ischemic area of the left ventricle was determined by means of the radioactive microsphere technique. Acute occlusion of the left anterior descending coronary artery produced a significant decrease of blood flow reaching the area irrigated by this artery; this decrease was of a lesser magnitude after administration of nitroglycerin. In addition, ischemia produced a disproportionate decrease in endocardial blood flow. This decrement was also of a lesser magnitude following administration of nitroglycerin. Blood perfusion to the non-ischemic myocardium was not altered. These results indicate that an intravenous bolus of nitroglycerin, given after a brief coronary occlusion simulating a coronary spasm, increases blood flow to the ischemic myocardium, induces a favorable redistribution of blood flow toward the ischemic endocardium and does not produce any decrement of blood perfusion to the non-ischemic myocardium.

Animals↗
PubMedDate not supplied

Seventh nerve spasm: effect of modification of cholinergic balance.

The seventh cranial nerve spasms of the face and the eyelids, including essential blepharospasm, are usually intractable and sometimes disabling. Deanol acetamidobenzoate was compared with the anticholinergic orphenadrine for relief of these spasms. Deanol cured two of 16 (12%) subjects and improved a third. Orphenadrine was much more effective, improving the conditions of 16 of 26 (62%) subjects between 57% and 100%. A crossover double-blind experiment showed high significance over a placebo.

Adult↗
PubMed1981

Tizanidine (DS 103-282) in the treatment of acute paravertebral muscle spasm: a controlled trial comparing tizanidine and diazepam.

In a double-blind trial tizanidine (DS 103-282: 5-Chloro-4-(2-imidazolin-2-yl-amino)-2,1,3-benzothiadiazole), a new muscle relaxant, was compared with diazepam in twenty in-patients suffering from acute paravertebral muscle spasm. All the patients had suffered from degenerative spinal disease for some years. Ten patients received tizanidine and the other ten received diazepam. Both drugs were administered t.i.d. for a period of 7 days. Significant improvements were reported in eight of the sixteen variables. Tizanidine was significantly superior to diazepam for two of these variables (lateral flexion of the lumbar spine, right and left). A centrally mediated blood-pressure-lowering effect was observed with both drugs. Tizanidine was generally better tolerated, only two patients reporting transient side-effects compared with five patients in the diazepam group. Like diazepam, tizanidine is a suitable drug for the treatment of acute paravertebral muscle spasm.

Acute Disease↗
PubMed2004

Infantile spasms in an infant with cytomegalovirus infection treated with ganciclovir.

A 3-month-old male infant with cytomegalovirus infection and intractable partial seizures was treated with ganciclovir for 6 weeks. The drug was well tolerated, and virus shedding in the cerebrospinal fluid and urine was eliminated, although infantile spasms at the age of 6 months appeared. At the age of 12 months, intractable seizures persisted, and the psychomotor development of the infant was markedly delayed. To our knowledge, no previous similar case has been reported. These findings suggest that treatment with ganciclovir of infants with cytomegalovirus infection results only in cessation of virus shedding in the cerebrospinal fluid and urine without having a preventive effect on the future appearance of infantile spasms. This may be due to the irreversibility of previous brain damage from the cytomegalovirus infection and the virostatic nature of the drug.

Anticonvulsants↗
PubMed2004

Infantile spasms: therapy and outcome.

Up-to date information about corticotropin (ACTH) in the treatment of infantile spasms and evaluation of the long-term outcome was provided to answer questions about (1) the efficacy of doses of ACTH in comparison with other drugs, especially with vigabatrin, and the efficacy in patients with tuberous sclerosis; (2) tolerability; and (3) long-term outcome. In two studies, high doses were not more effective than low doses but were more effective in another study. In the follow-up of the studies, there was no difference. In an open, randomized, prospective study, the efficacy and relapse rates of ACTH and vigabatrin treatment did not differ significantly. The high response rates in tuberous sclerosis complex were similar. Both drugs had severe side effects. In the long-term follow-up of 20 to 35 years, one third of the patients died, the intellectual outcome of the remaining patients was normal or slightly subnormal, and one quarter and one third of the patients were seizure free. ACTH should be the first choice for treatment of infantile spasms. The side effects of ACTH, unlike those of vigabatrin, are well known, treatable, and reversible. However, an open, prospective study to compare the efficacy, relapse rate, and long-term outcome of treatment with ACTH and vigabatrin is urgently needed. The frequency of visual field defects after vigabatrin therapy should be evaluated.

Adrenocorticotropic Hormone↗
PubMed1986

Continuous infantile spasms as a form of status epilepticus.

An infant with congenital cytomegalovirus infection first developed seizures at six weeks of age. At 3 1/2 months of age, he developed continuous infantile spasms that lasted for more than an hour. This episode of status epilepticus was terminated by intravenous lorazepam and paraldehyde, and seizures were subsequently controlled for seven months by adrenocorticotropic hormone (ACTH), valproic acid, and phenobarbital. This case demonstrates that continuous infantile spasms may occur as a unique form of status epilepticus in young infants.

Cytomegalovirus Infections↗
PubMed1990

Cushing's ulcer as a consequence of adrenocorticotropic hormone administration for infantile spasms.

Adrenocorticotropic hormone (ACTH) is the treatment of choice for infantile spasms despite its multiple side effects. This case represents a previously unreported side effect, peptic ulceration, occurring during ACTH treatment of infantile spasms. Two pathophysiologic mechanisms are proposed and examined. A direct effect of ACTH on the duodenum is considered but deemed unlikely. Alternatively, the combination of the patient's communicating hydrocephalus and ACTH's central effects is proposed to have led to increased intracranial pressure and a Cushing's ulcer. The need for pretreatment neuroimaging and H2-receptor antagonist use during ACTH therapy in all cases, along with gastrin level measurements in selected cases, is recommended.

Adrenocorticotropic Hormone↗
PubMed1996

Combination therapy of infantile spasms with high-dose pyridoxal phosphate and low-dose corticotropin.

A new combination therapy, high-dose pyridoxal phosphate (40 to 50 mg/kg daily) and low-dose corticotropin (0.01 mg [0.4 IU]/kg daily), was tried in 28 children with infantile spasms. Monotherapy with pyridoxal phosphate provided excellent seizure control in three (11%) of the 28 subjects. Corticotropin was subsequently added to the regimen of the remaining 25 patients. At 1 month after discontinuing corticotropin, 21 (84%) of the 25 patients experienced no seizures, and 22 (88%) of the 25 showed improvement in their electroencephalographic findings. The mean interval until achievement of seizure control was 4.1 days after the initiation of corticotropin. The outcome in the 21 patients has been followed for a mean period of 34.9 months (range, 2 to 81 months). Of these 21 patients, six (29%) have had relapses of infantile spasms, and 10 (48%) have experienced normal development. Transient increases in liver enzymes occurred in 14 (50%) of the 28 patients, but none of the patients developed more serious side effects. The investigators conclude that combination therapy with high-dose pyridoxal phosphate and low-dose corticotropin is a promising new therapy.

Adrenocorticotropic Hormone↗
PubMed1994

Oculogyric spasm in Asian psychiatric in-patients on maintenance medication.

BACKGROUND: The objective was to investigate the occurrence and characteristics of oculogyric spasm (OGS) in an Asian country. METHOD: All 2035 Asian (88% Chinese, 7% Malays and 5% Indonesians) psychiatric in-patients in the state psychiatric hospital in Singapore were surveyed for occurrence of oculogyric spasm (OGS) over a two-month period. RESULTS: Thirty-four patients (1.7%) developed OGS (53% male and 47% female). All the 34 patients had been on maintenance antipsychotic drugs for more than five months. Eighteen patients had recurrent attacks. The mean chlorpromazine equivalent daily dose for those patients with recurrent OGS was 511 mg. This was significantly higher (P < 0.05) than the 277 mg daily dose received by those without recurrent OGS. Most (68%) of the attacks occurred between 1400-2000 h suggesting that OGS may have a diurnal variation. CONCLUSIONS: OGS presenting as tardive dystonia may be due to a relative increase in cholinergic activity.

Adult↗
PubMed2004

Trigeminal afferent input alters the excitability of facial motoneurons in hemifacial spasm.

OBJECTIVE: To investigate whether skin or muscle afferent input via the trigeminal nerve alters the excitability of facial motoneurons in hemifacial spasm (HFS). METHODS: Botulinum toxin type A (BTX) was injected only to the orbicularis oculi (O. oculi) muscle of 21 patients with idiopathic HFS, and the excitability of the orbicularis oris (O. oris) motoneurons was monitored. The synkinetic response (SR) of the blink reflex and abnormal muscle response (AMR) were recorded from the O. oris before and after treatment. RESULTS: BTX injections produced marked to moderate improvement in the O. oculi of all 21 patients and in the O. oris of 17 (81%). The rectified areas of SR1 and SR2 were smaller after treatment. In particular, the AMR area showed a reduction (p = 0.02). CONCLUSIONS: The significant lessening of spasms in the O. oris after BTX injection to the O. oculi and the concomitant reduction in excitability of O. oris neurons are consistent with the hypothesis that in HFS, skin or muscle afferent volleys via the trigeminal nerve enhance the excitability of facial nerve motoneurons.

Action Potentials↗
PubMed2005

Infantile spasms and intellectual outcomes in children with tuberous sclerosis complex.

OBJECTIVE: To assess intellectual outcomes in a clinic-based population of patients with tuberous sclerosis complex (TSC) who also have a history of infantile spasms (IS) and to identify clinical risk factors for mental retardation in these patients. METHODS: This is a retrospective study of 50 patients with TSC and IS seen consecutively at the Massachusetts General Hospital Tuberous Sclerosis Comprehensive Clinic from December 2001 to October 2003. Data were obtained by chart review and by interview with patients' parents. RESULTS: Thirty-two (64%) of 50 patients with TSC with IS were found to have mental retardation (IQ or developmental quotient < 70). Three clinical variables showed an association with mental retardation: increased duration of IS from clinical onset to cessation (odds ratio [OR] per 1-month interval 1.09, 95% CI: 1.03 to 1.15, p = 0.004), increased time from treatment initiation until IS cessation (OR 1.07, 95% CI: 1.01 to 1.14, p = 0.020), and poor control of other seizures after IS (OR 17.76, 95% CI: 3.47 to 129.1, p = 0.00004). The following variables did not show an association with intellectual outcome: gender, initial seizure type, age at IS onset, or time from IS onset to treatment initiation. CONCLUSIONS: In patients with tuberous sclerosis complex who also have a history of infantile spasms (IS), the rate of mental retardation may be lower than previously reported. The risk of mental retardation increases significantly with prolonged duration of IS, prolonged time from treatment initiation until the cessation of IS, and poor control of subsequent seizures after IS.

Adolescent↗
PubMed1976

Misdiagnosis of spasm of the near reflex.

A bilateral sixth nerve palsy portends serious disease of the central nervous system and precipitates extensive patient studies. Spasm of the near reflex, characterized by intermittent convergence, accommodation, and miosis, is a functional disturbance. Five patients with hysterical spasm of the near reflex erroneously diagnosed as a bilateral sixth nerve palsy are reported. The pupillary sign, intense miosis on attempted lateral gaze, is emphasized as an important clue to the correct diagnosis. Despite extensive investigation, no disease of the central nervous system was found. Neurotic or hysterical features were evident in every patient.

Abducens Nerve↗
PubMed1978

A syndrome of progressive muscle spasm, alopecia, and diarrhea.

A syndrome of progressive muscle spasm, alopecia, and diarrhea was seen in 15 patients. The syndrome was characterized by painful intermittent muscle spasm, alopecia, amenorrhea, and malabsorption, and was sometimes associated with epiphyseal destruction and retarded growth. Symptoms began at age 10 and were more common in women than men. Muscle cramps affected the limbs first and then, several years after onset, the neck, trunk, and masticatory muscles. The course was progressive and led to malnutrition. Four patients died from 5 to 18 years after onset. Autopsy revealed polypoid changes throughout the gastrointestinal tract.

Adolescent↗
PubMed1979

Stimulus-induced EEG complexes and motor spasms in subacute sclerosing panencephalitis.

Patients with subacute sclerosing panencephalitis (SSPE) typically present with spontaneous periodic, stereotyped electroencephalographic (EEG) complexes and motor spasms, which are not usually affected by sensory stimuli. This report describes two patients with SSPE in whom the EEG complexes and the motor movements occurred mainly in response to afferent stimuli. Various forms of stimuli were effective in precipitating the movements and EEG complexes, particularly when the stimulus was unexpected or startled the patient. During sleep, the EEG complexes spontaneously occurred in a more periodic fashion, typical of SSPE, but afferent stimuli were still effective in evoking the EEG complexes and associated motor spasms.

Adolescent↗
PubMed1984

Pathophysiology of hemifacial spasm: I. Ephaptic transmission and ectopic excitation.

We studied 62 patients with hemifacial spasm to test the presence of ephaptic transmission and ectopic excitation. The zygomatic and mandibular branches of the facial nerve were stimulated separately, recording simultaneously from the orbicularis oculi and mental muscles. Antidromic impulses were transmitted bidirectionally between the two branches. Transmission took place in a fraction of slow conducting motor nerve fibers. After-activity and late-activity were recorded as single potentials or trains, suggesting autoexcitation of fibers. The interspike frequency was 250 to 350 Hz. Hyperventilation produced synchronous clonic-tonic activity, suggesting ectopic excitation caused by hypocalcemia. Ectopic excitation and ephaptic transmission are important pathophysiologic factors in hemifacial spasm.

Adult↗
PubMed1984

Tic convulsif, the combination of geniculate neuralgia and hemifacial spasm relieved by vascular decompression.

A patient had combined otalgia and intractable unilateral facial spasm, relieved by microsurgical vascular decompression of the seventh and eighth cranial nerve complex in the cerebellopontine angle without section of the intermediate nerve. A dolicho-ectatic anterior inferior cerebellar artery compressed the seventh and eighth cranial nerves complex, suggesting that vascular compression of the intermediate nerve or of the sensory portion of the facial nerve may cause geniculate neuralgia. "Tic convulsif" seems to be a combination of geniculate neuralgia and hemifacial spasm. This combination could be due to vascular compression of the sensory and motor components of the facial nerve at their junction with the brainstem.

Adult↗
PubMed1985

Vascular compression, hemifacial spasm, and multiple cranial neuropathy.

Hemifacial spasm is usually an isolated symptom resulting from facial nerve root compression. Three patients had, in addition, tinnitus, hearing loss, facial sensory loss, diminished gag reflex, dysphagia, and dysarthria. Acoustic reflexes were abnormal, and facial nerve conduction studies showed evidence of ephaptic transmission and ectopic excitation. Brain CT and metrizamide cisternography were normal. Surgical exploration showed compression of cranial nerve roots by posterior inferior cerebellar artery branches. After decompression, symptoms abated, and electrical signs of hemifacial spasm disappeared. Vascular compression of nerve roots in the cerebellopontine recess may cause multiple cranial neuropathy.

Aged↗