The march of focal motor epilepsy.
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Epilepsies are a diverse group of disorders with both shared and distinct features. Classification allows a coherent, systematic approach and serves as a 'universal language' between neurologists all over the world. There are two systems of classifications of epilepsies in use today. The International League Against Epilepsy (ILAE) Classification of Epileptic Seizures 1981 divides seizures into 3 morphologic types--generalised, partial and unclassifiable, with subtypes of each. This system has the advantage that seizures can be classified relatively easily and choice of antiepileptic is dictated by seizure type. However, the same patient may have more than one type of seizure either together or sequentially and many patients show a distinct evolution of their illness. This paved the way for another system of classification--the ILAE Classification of Epilepsies & Epileptic Syndromes 1989, which is meant to supplement the previous classification. An epileptic syndrome is defined as an epileptic disorder characterised by a cluster of signs and symptoms occurring together. According to this system, epilepsies are divided into 4 broad groups--localisation related and generalised, undetermined whether localised or generalised and special syndromes. Within the generalised and localised groups, there are further subdivisions into idiopathic, cryptogenic or symptomatic. This classification is complex and many syndromes are not adequately defined. At initial presentation, it may not be possible to identify the syndrome. Only a small proportion of patients fit into specific syndromes and a large proportion fall into non specific categories. For the present then, classification of epilepsy is still being refined. A brief description of major childhood epileptic syndromes according to age at onset is presented.
In order to understand the complex functional organization of the motor system, it is essential to know the anatomical and functional connectivity among individual motor areas. Clinically, knowledge of these cortico-cortical connections is important to understand the rapid spread of epileptic discharges through the network underlying ictal motor manifestation. In humans, however, knowledge of neuronal in vivo connectivity has been limited. We recently reported a new method, 'cortico-cortical evoked potential (CCEP)', to electrically track the cortico-cortical connections by stimulating a part of the brain through subdural electrodes and recording the cortical evoked potentials that emanate from a distant region of the cortex via neuronal projections. We applied the CCEP methodology to investigate in vivo cortico-cortical connections between the lateral motor cortex [LMCx; sensorimotor (SM) and lateral premotor areas] and the medial motor cortex [MMCx; supplementary motor area proper (SMA), pre-SMA and foot SM]. Seven patients with intractable partial epilepsy were studied. These patients had chronic implantation of subdural electrodes covering part of the lateral and medial frontal areas. As a part of the routine pre-surgical evaluation, comprehensive cortical mapping was performed by electrical stimulation of the subdural electrodes, and the precise localization of the subdural electrodes was defined by MRI co-registration. Single-pulse electrical stimuli were delivered to MMCx (7 patients) and LMCx (4), and CCEPs time-locked to the stimuli were recorded by averaging electrocorticograms from LMCx and MMCx, respectively. Short-latency CCEPs were observed when stimulating MMCx and recording from LMCx (mean latency: 21.6 ms, range: 9-47 ms) and vice versa when stimulating LMCx and recording from MMCx (mean latency: 29.4 ms, range: 11-57 ms). In terms of the location of these stimulus sites and CCEP responses along the rostrocaudal axis, regression analysis revealed a consistent correlation between the sites of stimulation and maximum CCEP for stimulation of both MMCx and LMCx. Functionally, stimulation of the positive motor areas in MMCx elicited CCEPs at the somatotopically homologous regions in LMCx (71%). The same findings were observed in MMCx (82%) upon stimulation of LMCx. In four subjects in whom bi-directional connectivity was investigated by stimulating both MMCx and LMCx, reciprocality was observed in the majority of connections (78-94%). In conclusion, the present study demonstrated a human motor cortico-cortical network connecting (i) anatomically homologous areas of LMCx and MMCx along the rostrocaudal cognitive-motor gradient; and (ii) somatotopically homologous regions in LMCx and MMCx in a reciprocal manner.
Single-pulse transcranial magnetic stimulation (s-TMS) with recording of motor evoked potentials (MEPs) from thenar muscles of both hands was performed on 84 patients with cryptogenic partial epilepsy and 50 healthy controls. We analyzed the cortical latency (CL), central conduction time (CCT), and threshold intensity (TI) required to elicit liminal MEPs at rest. In the patients, CL and CCT were normal, but TI was significantly higher than in the controls. Of the 84 patients, 65 were taking one or more antiepileptic drugs and 19 were untreated. The untreated patients had a significantly lower TI than the treated patients. In the treated patients, the TI increase paralleled the number of drugs taken. Additionally, in 2 subgroups of patients undergoing major modifications of antiepileptic treatment, TI dropped after partial withdrawal of medication and increased following the commencement of therapy. The results suggest that anticonvulsants depress the excitability of human motor pathways in epileptic subjects.
Palinopsia and motor partial seizures were associated in a 51-year-old man. The manifestation appeared six months after a right parietal hemorrhage. The relationship between the hallucinatory phenomena and epilepsy is discussed.
Motor cortex excitability was analyzed with transcranial stimulation in a patient with motor focal epilepsy and cortical myoclonus originating from the right motor cortex. The motor threshold to single transcranial magnetic shocks, but not to electric stimuli, was higher in the epileptic motor cortex than the normal left motor cortex. Single magnetic shocks elicited a short cortical silent period (50 ms) in the epileptic motor cortex. Paired magnetic stimuli also showed reduced cortico-cortical inhibition. These findings reveal an asymmetry in cortical excitability presumably due to impaired inhibition in the epileptic motor cortex.
PURPOSE: Unilateral seizures, or hemiconvulsive attacks, are motor seizures with tonic and/or clonic phenomena that involve only one side of the body. METHODS: We describe three adolescents who presented with hemiconvulsive seizures and were found to have 3-cps generalized spike-and-wave discharges on ictal and/or interictal EEG. All had normal neuroimaging studies. Two patients had been previously treated with carbamazepine, which led to a partial response in one patient. RESULTS: All three patients, however, are now seizure free on either sodium valproate or a combination of sodium valproate and lamotrigine. We believe the electroclinical diagnosis is that of idiopathic generalized epilepsy. CONCLUSIONS: Idiopathic generalized epilepsy presenting with hemiconvulsive seizures has not, to our knowledge, been previously described. However, the correct diagnosis of an idiopathic generalized seizure disorder, as opposed to a partial seizure disorder, has important treatment implications. The possible mechanism of hemiconvulsive seizures in idiopathic generalized epilepsy is discussed.
A patient with an unusual association between tuberous sclerosis complex and hemimegalencephaly is reported. At 2 days of age, CT scan disclosed right hemimegalencephaly with periventricular faint calcification. At 1 month of age, magnetic resonance imaging showed right hemimegalencephaly with T1 and T2 shortening. The right frontal and parietal lobes were compatible with transmantle dysplasia. At 6 months of age, the right hemisphere had decreased in size. Atrophic changes continued to progress until 3 years of age. Periventricular calcification in the right hemisphere became marked with age. The patient developed frequent partial motor seizures from 6 weeks of age and infantile spasms from 5 months of age. Electroencephalogram revealed low amplitude of background activities in the right frontal area. The cause of this atrophic change in the hemimegalencephalic hemisphere, which could be a hamartomatous lesion, remains unknown.
PURPOSE: Focal negative motor (akinetic) seizures are rare ictal events that are diagnostically challenging because they are difficult to differentiate from postictal Todd paresis, transient ischemic attacks, migraine events, and psychogenic episodes. METHODS: We describe a 45-year-old man in whom, after surgical drainage of a right frontoparietal subdural hematoma, prolonged episodes developed with flaccid paralysis of the left arm. RESULTS: A video-EEG recording demonstrated a close relation between the focal motor impairment and a clear-cut epileptic ictal discharge involving the right perirolandic cortical areas. CONCLUSIONS: On the basis of the electroclinical data, we hypothesize the involvement of the primary motor area in the genesis of the epileptic discharge triggering focal negative seizures.
Surgical management and strategies for the supplementary motor area (SMA) epilepsy are described. The following is our preoperative evaluations. The steps include functional magnetic resonance imaging (fMRI), interictal dipole tracing (DT), subdural electrodes mapping, measurements of movement-related cortical potential (MRCP), and the use of the intraoperative open MRI under conscious craniotomy. Six patients with SMA epilepsy underwent surgery after the mapping procedures and are now seizure-free. Combinations of preoperative (fMRI, subdural electrodes mapping) and intraoperative mapping allow exact localization and identification of the critical functional areas. Early postoperative deficits in motor and speech function were profound but patients recovered rapidly. It is concluded that the step of mapping procedures plays an important role in the management of SMA epilepsy surgery.
An epidemiologic survey was carried out by a team of medical doctors working in the Kozah district of North Togo during April 1987. It was designed to evaluate the incidence of epilepsy and cysticercosis, taking a sample population of 5,264 subjects aged over 15 years old. 81.7% of the population feed on pigs which are allowed to roam freely in the vicinity of people's habitations. Cysticercosis (123 cases; incidence 23.3 cases per thousand) was diagnosed when one of the following three tests gave a positive result:--serum sample greater than or equal to 0.400 OD with ELISA test (101 cas),--identification of a cysticercosis cyst on anatomopathological examination of subcutaneous cysts (12 cases),--presence of typical calcification patterns revealed by cranial or muscle X-rays (21 cases in 18 patients). General epilepsy or partial motor fits were entirely clinically diagnosed (88 cases; 16 per thousand of population). Cysticercosis proved to be the cause of 29.5% epileptic sufferers, onset occurring after the age of 50 in 66% of these patients.
Sleep starts, also called hypnagogic or hypnic jerks, are bilateral, sometimes asymmetric, usually single, brief body jerks that coincide with sleep onset. We describe sleep starts occurring repetitively in three epileptic children with spastic-dystonic diplegia and mental retardation. Repetitive sleep starts began at age 18 months in two children and at 9 months in the third. All three children had had feto-neonatal asphyxia; two presented with spastic and one with dystonic tetraparesis. One had West syndrome and two had partial motor seizures in the first year of life. Seizures were controlled in all three patients by antiepileptic drug therapy. Video/EEG recordings of all the children during the afternoon nap revealed clusters of sleep starts during the transition between wakefulness and sleep. Cluster lasted 4-15 min and comprised from twenty to twenty-nine contractions. The EEG counterpart of the event sometimes showed an arousal response, at times inducing complete awakening. Repetitive sleep starts should be recognized and clearly differentiated from epileptic seizures, especially if they appear in epileptic subjects. In neurologically compromised patients, they could represent an intensification of an otherwise normal event, due to the lack of strong inhibitory influence of the pyramidal tract resulting from the pyramidal lesion.
Changes in cerebral perfusion were studied during nine short-lasting simple partial motor seizures (SPS) in an 11-year-old girl. Blood flow velocity changes in both middle cerebral arteries (MCAs) were assessed by transcranial Doppler sonography during simultaneous EEG monitoring. Within 7.4 +/- 1.4 s after electroencephalographic seizure onset, flow velocity in the MCA ipsilateral to the electrical discharges started to increase and then gradually rose up to 70% above baseline values. Spread of the epileptic activity to the other hemisphere in the late stage of seizure was associated with a slight increase in blood flow velocity (<30%) in the contralateral MCA. After the end of the seizure, flow velocities returned to baseline within 47 +/- 7 s. Our findings indicate that focal epileptic seizures evoke asymmetric perfusion increases which are closely related to the onset and cessation of the electroencephalographic seizure activity.
BACKGROUND: Following an ischemic brain lesion, the affected cortex undergoes structural and functional changes that may lead to increased cortical excitability or decreased inhibitory neuronal activity, resulting in the occurrence of poststroke epileptic seizures in 6 to 10% of patients with stroke. METHODS: To assess motor cortical excitability, transcranial magnetic stimulation (TMS) was used to determine the silent period (SP) duration in 84 consecutive patients with ischemic stroke. RESULTS: In a subpopulation of six patients (38 to 72 years old) a significant decrease of the SP duration (mean 116 +/- 14 msec) was detected in either the arm or the leg on the affected side as compared to the corresponding unaffected limb (mean 231 +/- 32 msec). This electrophysiologic abnormality was clinically associated with focal motor seizures in five of the six patients, whereas none of the other 76 patients with normal or prolonged SP durations developed seizures or epilepsy. CONCLUSIONS: Silent period shortening in this group reflects decreased inhibitory activity that may partly be related to functional or structural impairment of GABAergic interneurons. TMS may be of value for determining patients with stroke at risk for developing poststroke seizures.
Atypical antipsychotic agents, specifically those with a high hyposerotonergic activity such as clozapine and clothiapine, have been associated with de novo obsessive-compulsive symptoms. We report the case of a 16-year-old adolescent male with severe mental impairment and disruptive behaviour who developed a compulsive head and body turning disorder on clothiapine. Such a symptom had to be distinguished from epileptic partial seizures; it promptly disappeared with the drug discontinuation.
OBJECTIVE: To identify the pattern of intracranial structural lesions in developmentally normal children with partial motor seizures by computed tomography and to monitor the behavior of single ring enhancing lesion (SREL) after a period of time with or without treatment. METHODS: Consecutive developmentally normal children between one year and twelve years with partial motor seizures in a tertiary care referral Hospital. After clinical examination and appropriate investigation for tuberculosis and cysticercosis, CT scan was performed. In addition to anticonvulsants, children received antituberculous or anticysticercal therapy if indicated. Repeat CT was performed on children with SREL after 6 months. RESULTS: Computed tomography was abnormal in 102 (68%) children. Majority of the children (75) had SREL. The lesions were located in decreasing order of frequency in the parietal lobe (65), frontal lobe (7), occipital lobe (1), temporal lobe (1) and cerebellum (1). Repeat CT scan was performed on 50 of the 75 children with SREL. Among these, in 41 children who were only on antiepileptic therapy, the SREL had decreased in size in thirty-two whereas in the rest (9), there was no change in the size. CONCLUSION: Awareness of the existence of disappearing SREL lesions is essential to avoid unnecessary treatment with antituberculous or anticysticercal therapy and provides ample justification in treating with anticonvulsant drugs only.
Two types of intracellular activity have been found in neurons in the cortical penicillin seizure focus during an interictal spike; a prolonged depolarization (PDS) followed by hyperpolarization or predominantly hyperpolarization. In the present study of a penicillin focus we have correlated the penicillin focus location with motor behavior and the cellular physiology with cellular morphology and location. Penicillin injection at the same location in the anterior sigmoid gyrus invariably resulted in focal seizures involving the contralateral shoulder. From the locations of the myoclonus and the focus, the character of the movements and the route of seizure spread we conclude that the same pathways mediate movement produced by penicillin seizures or by electrically stimulating the same motor cortex. Intracellular recordings and dye-marking of cells in and around the seizure focus revealed that PDS neurons were located within a 3 mm radius from the site of penicillin injection. All PDS neurons either lacked well filled processes or had abnormal dendrites. Inhibited neurons were all pyramidal neurons with normal dendrites. The dendritic abnormalities observed could be important in the genesis of the PDS.
Using functional magnetic resonance imaging (fMRI) we were able to observe, in detail, ictal brain hemodynamics during epileptic seizure caused by a brain tumor. A 53-year-old man was experencing partial motor seizures of the left side of his face and neck. In a brain MR image a mass lesion was found in the subcortical area of the right frontal lobe. We found focal spikes in his right hemisphere, though dominantly in C4 and T4 regions. fMRI investigations were carried out at 1.5 T (GE Signa Horizon) using gradient-echo echo-planar neuroimaging. We were able to perform the ictal examination twice. The activated regions were focalized and clearly found only on the lateral side of the tumor base. The region was in agreement with the epileptic focus examined using an electrocorticogram (ECOG). The signal intensity in the seizure focus rapidly increased 30 seconds before the convulsion was observed. After the end of the convulsion it also took 30 seconds to restore the signal intensity to the baseline value. fMRI is a very useful tool for various studies such as the identification of the epileptic focus, the mechanism of epileptic seizure, and so on.