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PubMed1995

Hemifacial spasm: successful treatment with felbamate.

Medical treatment of hemifacial spasm has generally been ineffectual. This report describes a 36-year-old man with a 7-year history of right hemifacial spasm who responded to felbamate (Felbatol) at doses of 1800-2800 mg per day. During treatment, he was able to achieve complete control of all spontaneous facial muscle spasms at rest. Activated hemifacial spasms, initiated by chewing, speaking, smiling, or grimacing were reduced in frequency and intensity of contraction by 40%-50%. Spontaneous worsening of the hemifacial spasms occurred at a dose of 3600 mg/day. Control of the hemifacial spasm was regained at a lower dose. This is the first reported use of felbamate for the control of hemifacial spasm. Microvascular decompression of the facial nerve has been the only known effective treatment for this condition. Successful felbamate therapy would provide an alternative to those patients for whom surgery is not a treatment option.

Adult↗
PubMedDate not supplied

Short term effects of valproate on infantile spasms.

Although valproic acid (VPA) is used to treat infantile spasms, VPA's efficacy in infantile spasms has not been determined in a controlled study. This study evaluated the effect of VPA on infantile spasms in patients who had not responded to adrenocorticotropin (ACTH) and corticosteroid therapy. The hypotheses were tested using a double-blind, randomized controlled crossover design. Twenty-one patients were randomly assigned to either the baseline-valproate-placebo treatment or the baseline-placebo-valproate treatment groups. Based on a repeated measures analysis of variance test, the valproate group had lower total mean spasm frequency levels than the placebo group. However, this difference did not remain after the crossover; the difference was due to the initial administration of valproate and placebo. When the spasm index was analyzed, the valproate treatment was found to have lower mean spasm index scores than the baseline treatments (p less than 0.03). No short-term toxic effects were observed in any patient. We conclude that short-term VPA therapy has a beneficial effect even on chronic infantile spasm patients who have failed to respond to ACTH/corticosteroid therapy.

Clinical Trials as Topic↗
PubMed1998

Hemifacial spasm due to posterior fossa tumors: the impact of tumor location on electrophysiological findings.

Ephaptic transmission is one of the electrophysiological hallmarks of hemifacial spasm. It is generally accepted that in the majority of patients with idiopathic hemifacial spasm, microvascular compression of the facial nerve at the site where the nerve exits the brain stem is the underlying cause. Whether the actual site of the ephapse is at the site of the lesion or at a nuclear level due to hyperexcitability of the facial motor nucleus is still controversial. Rarely, hemifacial spasm may be due to space occupying lesions in the cerebellopontine angle or in the brain stem. We report the electrophysiological findings of four patients with hemifacial spasm due to extra-axial tumors in different locations of the posterior fossa. The location of the tumor was intrameatal in one patient, in the cerebellopontine angle in two patients and in the brain stem in another patient. Facial nerve motor neurographies including transcranial magnetic stimulation revealed abnormal findings in two patients. Selective stimulation of facial nerve branches demonstrated delayed (ephaptic) responses in all but one patient whose hemifacial spasm had disappeared after treatment with carbamazepine. The latencies of the delayed responses did not correlate with the tumor location. In sum, the site of ephaptic transmission cannot be reliably determined by latency measurements of the delayed response because of its variability which is probably caused by the different size and diameter of the axons participating in ephaptic transmission as well as by the extent of focal demyelination at the site of the lesion. A neuroradiological work up including MR imaging should be mandatory in all patients with hemifacial spasm because electrophysiological studies fail to differentiate between idiopathic and symptomatic hemifacial spasm.

Adult↗
PubMed2001

Spasms in clusters in epilepsies other than typical West syndrome.

Although spasms in clusters are one of the major characteristics of West syndrome (WS), there are a significant number of patients who show spasms in clusters but do not fit the standard pattern of WS. It is possible to divide these atypical cases into the following three groups. Group 1: refractory epilepsies beginning in early infancy, associated with atypical electroencephalographic (EEG) features; Group 2: generalized epilepsies with spasms in clusters at ages of 2-3 years or above; and Group 3: localization-related epilepsies with spasms in clusters. Ictal clinical and EEG findings of spasms in clusters in these atypical patients and also those in WS are similar. Patients in Group 1 often suffer from Aicardi syndrome, cortical malformations, early myoclonic encephalopathy and Ohtahara syndrome. Most patients in Group 2 suffer from Lennox-Gastaut syndrome and other generalized epilepsies such as severe epilepsy with multiple independent spike foci. A significant number of them had a history of WS. Small number of patients in Group 2 can be diagnosed as having late-onset WS or long-lasting WS. In Groups 1 and 3 patients, cortical mechanisms play a critical role in their pathophysiology. The presence of older patients with spasms in clusters might indicate not only developing process of the brain but also some selective dysfunction of the brain plays an important role in the occurrence of spasms in clusters. Investigations on these atypical patients can help the understanding of pathophysiological mechanisms of WS and its related epileptic syndromes.

Brain↗
PubMed1980

Overnight polygraphic study of agenesis of the corpus callosum with seizures resembling infantile spasms.

In a 4-month-old female with agenesis of the corpus callosum, seizures resembling infantile spasms were observed succeeding tonic-clonic seizures. Interictal EEG revealed hypsarrhythmia with an asynchronous pattern. Overnight sleep polygraphy was performed before, during and after ACTH therapy. The results were as follows: 1) Clinical seizures were observed only before ACTH therapy. The clinical seizures and the ictal discharges without any apparent clinical seizures occurred in all stages of wakefulness, REM sleep and NREM sleep. 2) The clinical seizures first began with the tonic-clonic seizures and were followed by seizures resembling infantile spasms. The seizures resembling infantile spasms did not appear singly. The ictal discharges in the tonic-clonic seizures appeared only in one hemisphere and, moreover, asynchronously on many occasions. The polygram of a seizure resembling infantile spasms was just like that of infantile spasms. 3) Before ACTH therapy, decrease of REM sleep time and lack of slow wave sleep were found. Decrease of REM sleep time, lightening of sleep and prolongation of awake time were observed during ACTH therapy as compared with those before the therapy. It was indicated that the seizures resembling infantile spasms in the present case differed considerably from infantile spasms. In addition, it was suggested that the asynchrony of hypsarrhythmia and the asymmetry of ictal discharges were not attributable to agenesis of the corpus callosum but dysfunction of a lower area than the corpus callosum.

Adrenocorticotropic Hormone↗
PubMed1987

Identification of infants at risk for infantile spasms by neonatal polygraphy.

Reevaluation of neonatal EEGs and polygraphic tracings of 40 infants with infantile spasms and/or hypsarrhythmia resulted in the constitution of a compound score for the identification of infants at risk for infantile spasms by neonatal EEG. The score comprises 8 distinct items: 2 concern behavioral characteristics, 6 abnormality of EEG background activity and paroxysmal events. A tracing registered at conceptional age 36 to 44 weeks (eventually up to 50 weeks) presenting at least 4 of these 8 items is scored positive for the risk of evolving hypsarrhythmia. In a prospective study the polygraphic tracings of 941 newborn infants were evaluated for risk: 18 infants suffering from perinatal distress and 7 newborns with malformations of the brain were scored positive and all 25 developed infantile spasms and/or hypsarrhythmia. One infant with later infantile spasms was missed by the scoring system. None of the remaining infants scored negative manifested infantile spasms. Thus, correct positive prognostication was 100% and false negative 0.1%. By conventional EEG 5 out of 8 patients with infantile spasms were correctly predicted. The high validity of the risk-score based on polygraphic tracing between conceptional age 36 to 44 weeks may allow pre-onset treatment preventing secondary mental deterioration due to hypsarrhythmia and infantile spasms.

Electroencephalography↗
PubMed2000

Ganaxolone for treating intractable infantile spasms: a multicenter, open-label, add-on trial.

This is a multicenter, open-label, add-on trial, investigating the safety and efficacy of ganaxolone (GNX) in a population of children with refractory infantile spasms, or with continuing seizures after a prior history of infantile spasms. A total of 20 children aged 7 months to 7 years were enrolled in this dose-escalation study, after baseline seizure frequencies were established. Concomitant antiepilepsy drugs were maintained throughout the study period. The dose of GNX was progressively increased to 36 mg/kg/d (or to the maximally tolerated dose) over a period of 4 weeks, then maintained for 8 weeks before tapering and discontinuation. Seizure diaries were maintained by the families, and spasm frequency was compared with the baseline period. The occurrence of adverse events was clinically monitored, and global evaluations of seizure severity and response to treatment were obtained. A total of 16 of the 20 subjects completed the study, 15 of whom had refractory infantile spasms at the time of study enrollment. Spasm frequency was reduced by at least 50% in 33% of these subjects, with an additional 33% experiencing some improvement (25-50% reduction in spasm frequency). Ganaxolone was well tolerated, and adverse events attributed to GNX were generally mild. Ganaxolone was safe and effective in treating this group of refractory infantile spasms patients in an open-label, add-on trial. Further investigation with randomized, controlled study design is warranted.

Anticonvulsants↗
PubMed2000

Hemifacial spasm or somatoform disorder--postexcitatory inhibition after transcranial magnetic cortical stimulation as a diagnostic tool.

Hemifacial spasm (HFS) presents a frequent movement disorder. It is thought to have an organic origin. It therefore has to be distinguished from other facial involuntary movements, especially psychogenic tics, because the therapeutic approach differs. The present study opted to evaluate the diagnostic value of the postexcitatory inhibition (pI) after transcranial magnetic stimulation (TMS). After stimulating the contralateral hemisphere with the conventional flat coil and recording from the mentalis muscle, in 10 healthy controls and 10 patients postexcitatory inhibition was determined. PI showed no side to side difference in healthy controls (96.9 + 12.7 ms right, 87.9 +/- 10.8 ms left side, interhemispheric difference 6.4 +/- 3.8 ms). In 8 patients with hemifacial spasm, the duration of pI on the non-affected side did not differ from the healthy controls (87.9+/-43.5 ms). During spasm, pI on the affected side shortened increasingly until no inhibition could be induced. Afterwards the spasm pI was prolonged significantly (up to 140 ms longer than opposite side) before returning to normal values. Two patients presented no side differences of pI during the "spasm". An emotional conflict situation could be evaluated, supporting the diagnosis of somatoform disorder. As postexcitatory inhibition is mainly due to cerebral mechanisms, the electrophysiological results of the study pointed to a cortical influence on the hemifacial spasm. TMS seems to be an electrophysiological tool which allows a differentiation between organic and psychogenic spasm and enables a different therapeutic approach.

Adult↗
PubMed2001

Clinical spectrum of epileptic spasms associated with cortical malformation.

The spectrum of clinico-electrical characteristics of epileptic spasms associated with cortical malformation was studied in detail. The subjects were 15 patients suffering from spasms and cortical malformation demonstrated by MRI. The types of cortical malformation causing spasms were various, including hemimegalencephaly, diffuse pachygyria, focal cortical dysplasia, and polymicrogyria. Ohtahara syndrome was diagnosed in 3 patients, and West syndrome in 8. Symptomatic localization-related epilepsy preceded West syndrome in 4 patients, and a transition from Ohtahara syndrome to West syndrome was observed in one. West syndrome was followed by symptomatic generalized epilepsy including Lennox-Gastaut syndrome in 4 patients. Nine patients showed a condition which was labeled "epilepsy with partial seizures and spasms" (EPS) and characterized by the coexistence of partial seizures and spasms, and multifocal epileptic discharges on EEG. Spasms occurred only as EPS in 5 patients. EPS appeared following Ohtahara syndrome or West syndrome in 4 patients, and showed a transition to symptomatic localization-related epilepsy in 4. However, EPS did not evolve into generalized epilepsy, and persisted until the time of last follow-up in 5 patients. Therefore, the clinico-electrical pictures of patients with spasms and cortical malformation were diverse and not always limited within those of typical generalized epilepsy.

Cerebral Cortex↗
PubMed2004

Relief of hemifacial spasm after radiosurgery for intracanalicular vestibular schwannoma.

Secondary hemifacial spasm due to vestibular schwannoma is very rare. This is the first reported case of hemifacial spasm responsive to gamma knife radiosurgery in a patient with an intracanalicular vestibular schwannoma. Both the resolution of the spasm as well as tumor growth control were achieved with a single session of gamma knife radiosurgery. We report a 49-year-old male patient with a 6-month history of right-sided hearing loss and hemifacial spasm. MR examination revealed an intracanalicular vestibular schwannoma. The patient was treated with radiosurgery and received 13 Gy to the 50 % isodose line. Tumor growth control was achieved and no change in the tumor volume was present at the last follow-up at 22 months. The hemifacial spasm completely resolved after one year. Surgical removal of the presumably causative mass lesion has been reported to be the sole treatment in secondary hemifacial spasm. This case report indicates that it may be responsive to gamma knife radiosurgery. Whether or not this might be a treatment option in selected refractory cases of hemifacial spasm remains to be defined.

Hemifacial Spasm↗
PubMed1986

Benign myoclonus of early infancy or benign non-epileptic infantile spasms.

Lombroso and Fejerman (1983) described a syndrome which shares with West syndrome the clinical features of flexion spasm with onset in early infancy. However the syndrome differs from West syndrome in the absence of mental and psycho-motor involvement and having a normal EEG during wakefulness and sleep. We report four cases of these benign spasms of early infancy with polygraphic recording of the spasms providing the following information: the spasms are clinically similar to those often observed in West syndrome; namely they are characterized by a short (2-4 sec.) tonic contraction, there are no significant changes of the EEG concomitant to the spasm, series of spasms can occur not only during the daytime but also during sleep and immediately after awakening. Differential diagnosis is discussed with West syndrome and benign myoclonic epilepsy in infancy and with non-epileptic syndromes. The authors propose to name this syndrome: Benign Non-Epileptic Infantile Spasms.

Electroencephalography↗
PubMed1996

F-wave in patients with hemifacial spasm: observations during microvascular decompression operations.

In patients with hemifacial spasm (HFS), the spasm is due to cross compression of the facial nerve by a blood vessel. There are currently two hypotheses how the cross compression can cause HFS: 1. the spasm is caused by ephaptic transmission and hyperexcitability at the site of compression; and 2. the spasm is caused by hyperexcitability in the facial motonucleus. In peripheral nerves, F-waves, which result from the backfiring of antidromically activated anterior horn cells, have been proposed as indices of anterior horn cell excitability. Enhancement of the F-waves in facial muscles also indicates increased excitability of the facial motonucleus. On the other hand, abnormal muscle response (AMR), which can be elicited by stimulating one branch of the facial nerve and recording electromyographically from muscles innervated by other branches of the facial nerve, is specific for patients with HFS. We have therefore measured the AMRs and the F-waves in the facial muscle of HFS patients under anesthesia in order to investigate the excitability of the facial motonucleus. We obtained facial nerve evoked electromyograms from 14 HFS patients during microvascular decompression (MVD) operation. The F-waves, obtained with surface electrodes from the mentalis muscle, were defined as the second response after the M-wave. The F-waves in facial muscles cannot usually be elicited during surgical anesthesia using inhalation anesthetics. However, the F-waves were elicited on the spasm side in 10 out of 14 patients with HFS and the F-waves disappeared after MVD under anesthesia, as the early responses (R1) of the blink reflex were elicited on the spasm side before MVD under anesthesia. The F-waves elicited during anesthesia were suppressed significantly, compared with those before MVD. These results suggest that excitability in facial motonucleus increased on the spasm side.

Electric Stimulation↗
PubMed1997

Hemifacial spasm caused by a hemangioma at the geniculate ganglion: case report.

OBJECTIVE AND IMPORTANCE: Hemifacial spasm is rarely caused by facial nerve lesions in the temporal bone. Intratemporal facial nerve hemangiomas may initially present as facial spasm. CLINICAL PRESENTATION: A 30-year-old woman developed right hemifacial spasm. Physicians observed slight weakness on the right side of her face, in addition to the hemifacial spasm, but routine radiological examinations did not detect any abnormal findings along the course of the facial nerve. Although the patient underwent neurovascular decompression, the spasm persisted postoperatively. Two years after surgery, the right facial palsy progressed. Concurrently, the hemifacial spasm diminished. High-resolution computed tomography demonstrated a small mass lesion expanding the cortex of the right petrosal bone involving the geniculate ganglion of the facial nerve. INTERVENTION: The patient underwent a second craniotomy through a subtemporal extradural route, and the tumor was completely removed. A pathological examination demonstrated a cavernous hemangioma. CONCLUSION: Routine radiological examinations may fail to detect small intratemporal facial nerve hemangiomas, particularly at the geniculate ganglion. Therefore, when physicians encounter atypical facial spasm, the intratemporal portion of the facial nerve should be carefully examined using high-resolution computed tomography.

Adult↗
PubMed2004

Dynamic cortical activity during spasms in three patients with West syndrome: a multichannel near-infrared spectroscopic topography study.

PURPOSE: To investigate spatial and temporal cortical activity during clusters of naturally occurring epileptic spasms in patients with West syndrome (WS) by using multichannel near-infrared spectroscopy (mNIRS). METHODS: Conventional magnetic resonance imaging (MRI) and interictal and ictal single-photon emission computed tomography (SPECT) were carried out in three patients with WS. Thereafter, cortical hemodynamics during naturally occurring epileptic spasms were measured by mNIRS with simultaneous video/electroencephalographic (EEG) monitoring. RESULTS: Ictal SPECT revealed multiple hyperperfused areas within the cortex. With the use of mNIRS, an increase in regional cerebral blood volume (CBV) was observed in these areas, which is representative of cortical activation. The increase in CBV was accompanied by an increase in the concentrations of both oxy- and deoxyhemoglobin. The following heterogeneous regional changes in CBV during ictus were observed: (a) transient increases that were synchronized with spasms; (b) a gradual increase during an ictal event that fluctuated in synchrony with spasms; and (c) a combination of transient and gradual increases. An increase in regional CBV occurred in multiple areas that were activated either simultaneously or sequentially during an ictal event. Topographic changes in CBV were closely correlated with the phenotype of the spasm. CONCLUSIONS: During ictal events, multiple cortical areas were activated simultaneously or sequentially. The pattern of cortical activation closely affected the phenotype of the spasm, which suggested that the cortex was involved in the generation of spasms.

Brain Mapping↗
PubMed1981

Long-term prognosis after infantile spasms: a statistical study of prognostic factors in 200 cases.

A follow-up study was made on 200 children (115 boys, 85 girls) who had had infantile spasms, in order to compare their present condition over the age of six years with various prognostic factors. 48 of the children (30 males and 18 females) had died, and all the rest were aged six years or older at the time of final follow-up. 139 of the children had received ACTH therapy: at final follow-up, spasms had ceased in 43.5 per cent, and about the same proportion showed normal physical development; 23 per cent had normal mental development and 15.4 per cent were attending ordinary schools. Complete recovery (normal mental and physical development and attending ordinary schools) was achieved in only 19 cases (9.5 per cent). Of the cryptogenic cases, 44.4 per cent had made a full recovery. The poor prognostic factors for continuing seizures were evolution into other types of fits, relapse of seizures after ACTH therapy, seizures concomitant with spasms, and convulsions before the onset of spasms. Poor prognostic factors for physical development were delayed development before the onset of spasms, neurological abnormalities, PEG abnormality, symptomatic aetiology, neonatal convulsions, low birthweight, perinatal asphyxia and being female. Poor prognostic factors for mental development were delayed development before the onset of spasms, neurological abnormalities, PEG abnormality, prenatal and perinatal aetiology, relapse after initial ACTH therapy, laughing attacks, and evolution into other types of fits. Only in the cryptogenic cases was there significant correlation between the delay in treatment and the long-term prognosis for mental development. Poor prognostic factors for educability were very similar to those for mental development. In spite of conflicting views as to the long-term effects of ACTH, prompt treatment seems to be mandatory, at least in cryptogenic cases of infantile spasms.

Achievement↗
PubMed1995

Epileptic spasms in older children: persistence beyond infancy.

Although infantile spasms (IS) constitute a well-recognized epileptic syndrome, only recently did investigators propose that spasms be classified as a distinct seizure type, characterized by axial flexion/extension jerks in clusters. Five older children (aged 4.5-14.2 years) who underwent video-EEG monitoring in 1992 in our epilepsy program for intractable mixed seizure disorder (cryptogenic 1, symptomatic 4) demonstrated flexor and extensor spasms in clusters. Seizure onset was from birth to 1.33 years. All 5 had spasms during infancy that continued as the children aged. Ictal EEG during spasms showed a brief high-amplitude delta burst followed by diffuse background attenuation or diffuse background decrease with superimposed rhythmic beta or alpha activity. Multiple other seizure types were present. Interictal EEGs were markedly abnormal and demonstrated slowing, multifocal spikes, generalized slow spike-wave, and polyspike-wave. Two children with spasms were unsuccessfully treated with ACTH, and 3 underwent corpus callosotomy. We conclude that spasms occur in older children with intractable mixed seizure disorders and may persist beyond infancy.

Age Factors↗
PubMed1996

Risk factors of infantile spasms compared with other seizures in children under 2 years of age.

To analyze the magnitude of the risk factors for infantile spasms, we evaluated the records of 80 children with infantile spasms, 474 children with other types of epilepsy, 2,196 children with febrile seizures, and 262 children with CNS infections. There was a family history of seizures in 13.8% of children with infaNtile spasms, 28.5% of children with other forms of epilepsy, 25.5% of children with febrile seizures, and 5.3% of children with CNS infections. Children with a family history of seizures were 2.82 times more likely to have infantile spasms, 7.05 time more likely to have other epilepsy, and 6.08 time more likely to have febrile seizures than controls (children with CNS infections). However, a family history of seizures increased the risk for infantile spasms only in the cryptogenic group. Children with infantile spasms were significantly more likely to have cerebral palsy, microcephaly, hydrocephaly, CNS malformations, neonatal hypoxia, or neonatal seizures than children with other types of epilepsy, febrile seizures, or CNS infections. There was a modest genetic predisposition to seizures in children with infantile spasms. However, our data suggest a much stronger association with underlying neurologic abnormalities, mainly neonatal seizures, neonatal hypoxia, and CNS malformations.

Age Factors↗
PubMed1998

Evidence of late-onset infantile spasms.

PURPOSE: To underline the unusual but possible occurrence of epileptic spasms (ES) in children >1 year of age. METHODS: Cases in whom onset of spasms occurred after 1 year of age were identified through a retrospective review of the records of all patients referred for ES to the Saint-Vincent de Paul Hôpital (Paris) and American Memorial Hospital (Reims) between 1974 and 1994. RESULTS: Eighteen cases were identified among the 734 children referred for ES, 18 cases were identified where spasm onset time ranged from 12 to 38 months of age. In 1/3 of the cases, the diagnosis was suspected from the onset of clinical manifestations; in the remaining 2/3, diagnosis was delayed by a mean 6 months (range, 2-25 months). Neurobehavioral regression affected two-thirds of the patients. Modified hypsarrhythmia was present in 11 patients; all but one exhibited major and diffuse spike- and slow-wave activity. EEG abnormalities were detected in the frontal areas in 11 patients. Spasms were cryptogenic in 9 patients. Steroids were administered to 13 patients; these controlled the spasms in 6 patients. Outcome was favorable for both seizures; cognition favorable in only 2 of the 18 patients. CONCLUSIONS: Beginning after the first year of life, ES, or late-onset infantile spasms, are distinct from early Lennox-Gastaut syndrome, although etiology, prognosis and treatment are similar to that for the classical infantile spasms.

Age of Onset↗