Treatment of the Lennox syndrome with ACTH: a clinical and electroencephalographic study.

In 45 cases of Lennox syndrome treated with ACTH, the immediate and long-term effects and the various factors affecting them were investigated by a follow-up study. 1) Regarding the immediate effect, 23 (51.1%) of the 45 cases became "seizure free" for over 10 days. 2) As to the long-term prognosis of these 23 cases, 10 cases relapsed into Lennox syndrome within 6 months and in the remaining 13 cases, seizures were suppressed for over 6 months; out of these 13, seizure relapse was observed in eight cases from 9 months to 7 years later, and the other five cases followed a very favorable course without relapse. 3) The favorable factors related to the effect of ACTH for Lennox syndrome are: a) age at ACTH treatment: up to 4 years old, b) time lag between onset of Lennox syndrome and initiation of ACTH treatment: the shorter the better; at least within 1 year, preferably within 3 months, c) presumptive causes and underlying diseases: idiopathic cases are best, d) mental defects before treatment: the slighter the better, e) seizure patterns: without tonic seizures, and f) EEG findings: disorganized diffuse slow spike-waves without asymmetry. 4) It is desirable to continue the ACTH treatment as long as possible, with the goal of the disappearance of seizure discharges, or at least the disappearance of diffuse seizure discharges.     

The perfusion defect seen with SPECT in West syndrome is not correlated with seizure prognosis or developmental outcome

We used interictal single photon emission computed tomography (SPECT) on 40 patients with West syndrome to determine whether cortical perfusion abnormalities are closely related to the development of West syndrome and whether they are correlated with the long-term seizure prognosis or the developmental outcome. Localized cortical perfusion abnormalities were seen in 24 patients (60%), while 15 patients (38%) were classified as normal. The remaining patient showed hyperperfusion of the basal ganglia bilaterally. Of 24 patients with localized perfusion abnormalities, unifocal cortical hypoperfusion was present in 11, multifocal hypoperfusion in 10, multiple cortical hypo- and hyperperfusion in one, hyperperfusion of the bilateral frontal cortices and brain stem in one, and focal hyperperfusion in the residual frontal cortex in one. For statistical analysis, we focused on 26 patients (cryptogenic; 10, symptomatic; 16), who were followed for more than 2 years after the onset of tonic spasms (mean 5.0 years). The results showed that focal cortical perfusion abnormalities were not correlated with the long-term seizure prognosis, the developmental outcome, or the response to ACTH therapy. In agreement with previous reports, the results of interictal SPECT suggested that focal cortical lesions play an important role in the development of West syndrome. However, statistical analysis showed that the existence of cortical dysfunction as defined by SPECT did not predict the seizure prognosis or the developmental outcome.     

Lennox–Gastaut syndrome in adulthood: Long-term clinical follow-up of 38 patients and analysis of their recorded seizures

Lennox–Gastaut syndrome (LGS) is a severe epileptic encephalopathy with childhood onset that usually continues through adolescence and into adulthood. In the long term, patients with this condition still have intractable seizures, intellectual disability, behavioral problems, and physical comorbidities. The aim of this study was to describe the clinical and EEG characteristics of a group of adults with Lennox–Gastaut syndrome. We identified 38 (22 females, 16 males) patients with LGS older than age 18 years at their last evaluation, with mean age of 43.3 ± 10.6 years. Median follow-up was 14.4 years (range: 2–40).All of our patients had 3 or more seizure types during their clinical history. The most prevalent seizure types at follow-up were atypical absences (28/38), tonic (28/38), generalized tonic–clonic (17/38), focal (11/38), and myoclonic seizures (9/38). All patients had drug-resistant seizures. Besides epilepsy, intellectual disability and behavioral problems were prominent features. Surprisingly, paroxysmal nonepileptic seizures were reported in 3 patients. Our observations confirm the poor outcome of Lennox–Gastaut syndrome through adulthood, regardless of age at seizure onset, etiology, and history of previous West syndrome.     

The Long-Term Prognosis of Minor Seizures in Childhood Epilepsy

The long–term clinical and electroence-phalographic follow-up studies were carried out for more than three to 13 years on 72 cases with minor seizures in childhood epilepsy at the Department of Pediatrics of Keio University from January 1963 to January 1973. Minor seizures were classified into typical absences, infantile spasms, Lennox syndrome, myoclonic seizures and atonic seizures. The results of each group were summarized as follows, but atonic seizures were excluded because there were too few cases of this group.

• 1 Some patients with typical absences were not preceded by other epileptic seizures, while some others had generalized tonic-clonic seizures, including febrile convulsions (mixed form by Fukuyama), prior to the onset of typical absences. Typical absences were not transformed into other epileptic seizures excluding generalized tonic-clonic seizures at follow-up. Twenty-one (84%) out of 25 cases with typical absences had ceased having seizures over one year at follow-up. Spontaneous, generalized spike and wave discharges including 3 c/s spike and wave complexes were recorded in about one half of all cases at the final interictal EEG recordings, but focal components were never seen. Mental retardation was very rare.
• 2 The attacks of infantile spasms had a tendency to disappear or be converted to variously other epileptic fits, excluding typical absences, with the development of age. EEG-findings were transformed into focal or Muse paroxysmal discharges and basic dysrhythmia from hyp-sarhythmia after many years, but classical 3 c/s spike and wave discharges were never seen. Moderately or severely mental retardation was found in most of these patients.
• 3 It was characteristic of Lennox syndrome that the improvements or successions of tonic seizures and atypical absences were repeated and diffuse slow spike and wave discharges were sustained in most of children during the periods of follow-up. Mental retardation was found in half of all cases.
• 4 Myoclonic seizures had ceased in most of children without transformations to other epileptic fits at follow-up, but some tendency toward relative dirsculty in control of clinical seizures was noted. Two thirds of these patients were of normal intelligence, while one third had retarded intelligence at follow-up.

Based on the above–mentioned results, the literatures concerning prognosis of various types of minor seizures in childhood epilepsy were studied.     

Prognosis of Epilepsy: A Preliminary Report of a Multi-Institutional Study in Japan

• 1. ) A multi–institutional study on the long–term prognosis of epilepsy was started in Japan in 1975 by a research team consisting of seven psychiatric and three pediatric institutions. The investigation of the outcome of epileptic seizures and EEG findings 10 years after the onset (subject: onset of illness later than 1961; first visit between 1964 and 1966), five years after the onset (subjects: onset later than 1966; first visit 1968—-71), three years after the onset (subjects: onset later than 1968; first visit 1970–73) were performed in September 1976.
• 2. ) The rate of the adult epileptic cases which were followed up for more than 10 years was 11.6%, for more than five years 23.9%, and those of children epileptics, 10-year follow-up 30.4%, five-year follow-up 50.6%. The rates of successful follow-up by means of mailing inquiries in adult and children cases were 53.3% and 56.2%, respectively.
• 3. ) The seizure disappeared in 55% and 68% of the adult cases with generalized convulsive seizures within five and 10 years, respectively. In the patients with psycho–motor seizures, the number of improved cases were almost equal to or slightly less than that of aggravated cases. Among the children cases, the outcome of cases with absence was the best (disappeared in 100%), worst in cases with West and Lennox syndromes (disappeared 63% and 50%, respectively), and those of generalized convulsive seizures (disappeared 82%) and psychomotor seizures (disappeared 65%) in between.
• 4. ) As for the clinico–electroencephalo–graphic correlations, about half of the adult and approximately 70% of the children cases showed a parallel change in clinical seizure frequency and EEG pictures. In most of the non–parallel cases, aggravation of EEG abnormalities was observed despite of the clinical improvement.
• 5. ) The methodological problems on the study of prognosis of patients with epilepsy were discussed.

This study was carried out by the Group for Study on the Prognosis of Epilepsy in Japan chaired by T. Okuma, M.D. Investigators collaborating in this study are listed as follows: T. Sato, M.D. and H. Fukushima, M.D., Hirosaki University Hospital; H. Kumashiro, M.D., K. Maruko, M.D., Y. Takahashi, M.D. and T. Ono, M.D., Fukushima Medical College Hospital; Y. Fukuyama, M.D., Tokyo Women's Medical College Hospital; T. Seki, M.D., Y. Kawahara, M.D. and M. Hirose, M.D., Keio University Hospital; S. Kamiya, M.D., K. Minami, M.D., S. Kita, M.D. and S. Okamoto, M.D., Kansai Medical College Hospital; S. Ohtahara, M.D., Y. Yamatogi, M.D., Y. Ohtsuka, M.D., E. Oka, M.D. and S. Kanda, M.D., Okayama University Hospital; K. Hosokawa, M.D. and T. Ku goh, M.D., ditto; H. Yamada, M.D., H. Yoshida, M.D. and H. Ninomiya, M.D., Kyushu University Hospital; s. U &, M.D., T. Shirakawa, M.D., Y. Nakazawa, M.D. and K. Inanaga, M.D., Kurume University Hospital.     

On the Lennox Syndrome with the Onset in Puberty

The cases of the Lennox syndrome which began at the age between 14 and 16, namely in puberty, were reported. The first case, a 22–year-old woman, was attacked with grand mal seizure at the age of 15, and one year later “Juchzer” (ref. Doose) appeared. This continued for two years, and then astatic seizures appeared. The second case, a 20–year-old woman, was attacked with grand mal seizure at the age of 14, and soon after astatic seizure appeared. Thus both are closely related with grand mal seizures. The first case is idiopathic, and the intelligence developed normally till the onset of the Lennox syndrome, but after that it became disturbed. The second case is residual state of arsenic toxicosis, and so the intelligence was already disturbed before the onset. On EEG both of them at first showed diffuse slow waves of high voltage, and in several years after the appearance of the Lennox syndrome, slow spike-waves were found. On therapy, scarcely no beneficial effects were noted by conventional antiepileptics, and by nitrazepam only the temporal beneficial effect was obtained. The symptoms of our cases are generally similar to the Lennox syndrome in infancy. Though we observed only two cases, we presented our cases as the Lennox syndrome with late onset.     

Epilepsy in patients with Cornelia de Lange syndrome: A clinical series

PurposeCornelia de Lange (CdLS) syndrome is characterized by multiple congenital anomalies and mental retardation. Epilepsy is a clinical feature found in about 20% of cases, but there are no data about its electroclinical features and long-term outcome.Methodswe describe a clinical series of fourteen Caucasian CdLS paediatric patients who developed epilepsy, with special reference to the long term prognosis.ResultsEpilepsy manifested between age 0.6 and 16.3 years. The majority of patients (64.3%) presented with partial seizures and interictal EEGs mainly revealed focal epileptic paroxysms involving temporal and parietal areas. Thirteen of 14 children became seizure-free with treatment. Valproate monotherapy was used in eight patients (57.1%), controlling seizures in seven. Otherwise monotherapy with topiramate, levetiracetam, carbamazepine and oxcarbazepine appeared to be effective in controlling seizures in four cases. At the end of the follow-up (age range, 7.3–24.2 years; follow-up, 8.2 ± 3.9 years), thirteen patients were seizure free (three still in therapy), while in one patient seizures were not controlled.ConclusionsPartial epilepsy is the most common type of epilepsy in CdLS patients. In the majority of cases the prognosis of this epilepsy is favourable and therapy can be withdrawn after few years of complete seizure control.     

Seizure and developmental prognosis of West syndrome--combination therapy with high-dose vitamin B6, valproate and low-dose ACTH]

Twenty patients with West syndrome were initially treated with high-dose vitamin B6 (40 to 50 mg/kg/day) and valproate (40 to 50 mg/kg/day). Three became seizure free. For the remaining 17 patients, low-dose synthetic ACTH (0.01 mg [0.4 IU]/kg/day) was added to the regimen. One month after the end of ACTH therapy, 13 patients were seizure free. Thus 16 patients in total(80%) were free of seizures(group A). The treatment was ineffective for the remaining 4 patients (20%; group B). During the following for a mean period of 64 months (range, 48 to 83 months), 9 in group A had a relapse of epileptic seizures. However, only 4 in this group had epileptic seizures at the end of the study (5-7 years of age), all of which were partial and infrequent. In group B, two had frequent intractable seizures, and one was seizure free at the end of the study. One died at the age of 1 year. In group A, 2 patients showed normal or subnormal mental development. Mild, moderate and severe mental retardation were seen in 3, 4 and 7 patients respectively. In group B, all patients showed severe mental retardation. In this study, the rate of evolution into intractable epilepsy was low, but long-term mental development was poor. Seizure control by itself seemed to be insufficient to improve long developmental prognosis of West syndrome.     

Landau–Kleffner syndrome: A study of 29 patients

PurposeThe aim of the study was to retrospectively analyze the electroclinical features, etiology, treatment, and prognosis of 29 patients with Landau–Kleffner syndrome (LKS) with a long-term follow-up.MethodsInclusion criteria were a diagnosis of LKS with: (1) acquired aphasia or verbal auditory aphasia; (2) with or without focal seizures, secondarily generalized tonic-clonic seizures, absences, or atonic seizures.ResultsMean follow-up was 12 years. All cases except six had seizures. Before the onset of aphasia, developmental language and behavioral disturbances were present in 19 and 14 patients, respectively. All patients had verbal auditory agnosia. Aphasia was severe in 24 patients and moderate in five. Nonlinguistic cognitive dysfunctions were moderate in 14 patients. Behavioral disturbances were found in 16 patients. During the continuous spike-and-wave discharges during slow sleep phase, the spike-wave index was >85% in 15, 50–85% in eight, and 30–50% in four. In two patients, the EEG recording showed occasional bilateral spikes, without continuous spike-and-wave discharges during slow sleep. In this phase, the awake EEG recording showed more frequent interictal abnormalities, predominantly in the temporal regions. Eight patients recovered language completely, but the remaining patients continue to have language deficits of different degrees.ConclusionLandau–Kleffner syndrome is an epileptic encephalopathy characterized by acquired verbal auditory aphasia and seizures in most of the patients associated with continuous or almost continuous spike-and-wave discharges during slow wave sleep. The most commonly used treatments were clobazam, ethosuximide, sulthiame. High-dose steroids were also administered. Adequate and early management may avoid language and cognitive deterioration.     

Vagus nerve stimulation vs. corpus callosotomy in the treatment of Lennox–Gastaut syndrome: A meta-analysis

PurposeLennox–Gastaut syndrome (LGS) is an epileptogenic disorder that arises in childhood and is typically characterized by multiple seizure types, slow spike-and-wave complexes on EEG and cognitive impairment. If medical treatment fails, patients can proceed to one of two palliative surgeries, vagus nerve stimulation (VNS) or corpus callosotomy (CC). Their relative seizure control rates in LGS have not been well studied. The purpose of this paper is to compare seizure reduction rates between VNS and CC in LGS using meta-analyses of published data.MethodsA systematic search of Pubmed, Ovidsp, and Cochrane was performed to find articles that met the following criteria: (1) prospective or retrospective study, (2) at least one patient diagnosed with Lennox–Gastaut syndrome, and (3) well-defined measure of seizure frequency reduction. Seizure reduction rates were divided into seizure subtypes, as well as total seizures, and categorized as 100%, >75%, and >50%. Patient groups were compared using chi-square tests for categorical variables and t-test for continuous measures. Pooled proportions with 95% confidence interval (95% CI) of seizure outcomes were estimated for total seizures and seizure subtypes using random effects methods.Results17 VNS and 9 CC studies met the criteria for inclusion. CC had a significantly better outcome than VNS for >50% atonic seizure reduction (80.0% [67.0–90.0%] vs. 54.1% [32.1–75.4%], p < 0.05) and for >75% atonic seizure reduction (70.0% [48.05–87.0%] vs. 26.3% [5.8–54.7%], p < 0.05). All other seizure types, as well as total number of seizures, showed no statistically significant difference between VNS and CC.ConclusionsCC may be more beneficial for LGS patients whose predominant disabling seizure type is atonic. For all other seizure types, VNS offers comparable rates to CC.     

Long-term follow-up study of West syndrome associated with tuberous sclerosis.

AIM: To analyze the evolution of seizures, electroencephalographic (EEG) features, seizure outcomes, and social outcomes, in order to formulate a basis for the long-term prognosis of West syndrome (WS) associated with tuberous sclerosis (TS). METHOD: The clinical profiles of 50 patients with TS, who presented with WS in infancy and had been monitored for more than 10 years after the initial examination, were analyzed. RESULTS: In 86% of the patients the seizure type did not change throughout the course of this study. However, 14% of patients had developed other types of seizures by the end of the follow-up period.The seizure outcome was unfavorable for patients with generalized seizures. Partial seizures were controlled in nine (64%) of the 14 patients, and normal or slightly subnormal intelligence accounted for seven of the 14 patients. CONCLUSIONS: Therefore, in patients with WS associated with TS, the seizure outcome and social activity depended on the type of seizure that developed during the course of this disorder. The outcome of patients with WS associated with TS seems to be better than that of WS in general.     

Predictive value of EEG findings at control of epileptic spasms for seizure relapse in patients with West syndrome

PurposeTo evaluate the prognostic importance of electroencephalography (EEG) findings at cessation of epileptic spasms for seizure outcome.MethodsWe reviewed 71 children with West syndrome (cryptogenic 14) who had obtained control of epileptic spasms with initial treatment (adrenocorticotropic hormone (ACTH) 37, high-dose vitamin B₆ 2, and antiepileptic drugs 32). According to the EEG findings at control of epileptic spasms, the subjects were divided into three groups: normal group (no epileptic activity, n = 12), abnormal group (residual epileptic activity without hypsarrhythmia, n = 53), and hypsarrhythmic group (persisting hypsarrhythmia, n = 6).ResultsOverall, 47 (66%) of the 71 patients (cryptogenic 4) had experienced relapses of seizures (epileptic spasms 23 and focal seizure 24) after initial control of epileptic spasms. Within symptomatic cases, seizure relapse rate varied widely from 0% (Down syndrome) to 100% (tuberous sclerosis), depending on underlying causes. Seizure relapse depended on the EEG findings at control of epileptic spasms. The normal group had a significantly lower seizure relapse rate (17%) in comparison with the abnormal group (75%), the hypsarrhythmic group (83%), and the epileptiform (abnormal plus hypsarrhythmic, 76%) group. No significant difference in seizure relapse rate was observed between non-hypsarrhythmic (normal plus abnormal, 65%) and hypsarrhythmic groups. At the last follow-up, normal group children also showed a favorable seizure prognosis (seizure control 100%).ConclusionsA favorable seizure prognosis is associated with the disappearance of epileptic activity, but not the resolution of hypsarrhythmic pattern on EEG at control of epileptic spasms. We suggest that effective treatment for West syndrome should produce both cessation of epileptic spasms and disappearance of epileptic activity on EEG.     

Hemispherectomy in pediatric patients with epilepsy: a study of 45 cases with special emphasis on epileptic syndromes

Objective

In this study we report the clinical outcomes of hemispherectomy for epilepsy in pediatric patients with special emphasis on the epileptic syndromes and their etiologies.

Material and methods

We retrospectively studied 45 patients with medically refractory epilepsy with hemispheric lesions who underwent hemispherectomy at the ??Hospital de Pediatría Prof. Dr. Juan P. Garrahan??, Buenos Aires, Argentina between February 1990 and February 2010. Patients had been assessed using a standard protocol involving clinical, neuroradiological, neurophysiological, and neuropsychological teams.

Results

Twenty-seven males and 18 females with a mean age of 8.5?years (range, 2?months to 18?years) who underwent epilepsy surgery for refractory epilepsy were assessed. The mean time of follow-up was 9.5?years (range, 1 to 16?years). The following epileptic syndromes were recognized: West syndrome in 15 patients (33.5%), Rasmussen syndrome in 13 (29%), focal symptomatic epilepsy in 8 (17.5%), startle epilepsy in 6 (13.5%), Lennox?CGastaut syndrome in 2 (5%), and continuous spikes and waves during slow sleep in 1 (2%). The surgical specimens revealed malformations of cortical development in 18 patients (40%), Rasmussen encephalitis in 13 (29%), porencephalic lesions in 10 (22%), gliosis in 2 (4.4%), tumor in 1 (2.2%), and Sturge?CWeber syndrome in 1 (2.2%).

Conclusion

The outcome of hemispherectomy in pediatric patients is good for those with refractory epilepsies, such as West syndrome, Lennox?CGastaut syndrome, epileptic encephalopathy with continuous spikes and waves during slow sleep, and startle epilepsy arising from a hemispheric lesion associated with hemiplegia.     

Aicardi Syndrome: A Longitudinal Clinical and Electroencephalographic Study

Summary: We report clinical and EEC follow-up of 6 children with Aicardi syndrome. Age at seizure onset was <3 months in 5 patients and 4 months in 1 patient. All patients had spasms, and these continued at time of follow-up in 5 patients. Five patients had seizures other than spasms which disappeared during early infancy. Bilateral independent bursts (BIBs) characteristic of Aicardi syndrome were noted in 4 patients. In 1, BIBs showed suppression-burst patterns. BIBs converted to hypsarrhythmia or multifocal spikes with a tendency to BIBs during sleep. BIBs disappeared completely during both waking and sleeping states between the ages of 3 years 1 month and 4 years 9 months. After BIB disappearance, the EEG of 1 patient showed diffuse slow spike-and-wave complexes most of which appeared asynchronously. At onset, 1 patient had early-infantile epileptic encephalopathy with suppression-burst which evolved initially into West syndrome (WS) and then Lennox-Gastaut syndrome (LGS). The other 3 children also had WS.     

Long-term follow-up study of Lennox-Gastaut syndrome in patients with severe motor and intellectual disabilities: with special reference to the problem of dysphagia.

A long-term follow-up study of Lennox-Gastaut syndrome (LGS) ( > 10 years) was conducted with 38 patients with severe motor and intellectual disabilities (SMID) to clarify the relationship between the rapid development of dysphagia and epileptic seizures, and to elucidate the long-term evolution of LGS in patients with SMID. Those who showed a relatively favourable seizure outcome were compared to those with a poor seizure outcome. Poor seizure outcome correlated strongly with: (a) an early appearance of dysphagia and additional deterioration of the already retarded mental function; (b) a predominance of atypical absence seizures; and (c) persistent frequent epileptiform discharges during electroencephalographic evaluations. Neither age at seizure onset nor intelligence level at the time of the last examination was correlated with seizure prognosis. Further, seizure prognosis was not related to the aetiology of LGS. Repeated seizures apparently caused development of progressive epileptic encephalopathy, in addition to the underlying severe brain damage. Since development of dysphagia burdens an already severely handicapped patient with intensive medical care, efforts to reduce the seizures and design a long-term care plan are of great importance.     

Delayed myelination at the onset of cryptogenic West syndrome

To evaluate the prognostic value of delayed myelination at the onset of cryptogenic West syndrome, the relationship between the seizure or developmental outcome and myelination was examined. Cranial magnetic resonance imaging studies were performed in nine cryptogenic cases. Infantile spasms were controlled in all patients, but three cases showed a mild developmental delay at 2 years after onset. Delayed myelination was observed in three cases (33.3%) on T(1)-weighted images and in two cases (22.2%) on T(2)-weighted images. In the present study, neither the seizure outcome nor developmental status was positively correlated with the existence of delayed myelination at the onset of cryptogenic West syndrome.     

Reduction of seizure frequency after epilepsy surgery in a patient with STXBP1 encephalopathy and clinical description of six novel mutation carriers

Mutations in STXBP1 have been identified in a subset of patients with early onset epileptic encephalopathy (EE), but the full phenotypic spectrum remains to be delineated. Therefore, we screened a cohort of 160 patients with an unexplained EE, including patients with early myoclonic encephalopathy (EME), Ohtahara syndrome, West syndrome, nonsyndromic EE with onset in the first year, and Lennox‐Gastaut syndrome (LGS). We found six de novo mutations in six patients presenting as Ohtahara syndrome (2/6, 33%), West syndrome (1/65, 2%), and nonsyndromic early onset EE (3/64, 5%). No mutations were found in LGS or EME. Only two of four mutation carriers with neonatal seizures had Ohtahara syndrome. Epileptic spasms were present in five of six patients. One patient with normal magnetic resonance imaging (MRI) but focal seizures underwent epilepsy surgery and seizure frequency dropped drastically. Neuropathology showed a focal cortical dysplasia type 1a. There is a need for additional neuropathologic studies to explore whether STXBP1 mutations can lead to structural brain abnormalities.     

A Long-Term Clinical Course of Psychomotor Epilepsy (I)

A comparative study of clinical features and outcome was undertaken in 21 epileptic patients, five suffering from isolated psy-chomotor seizure without generalized convulsion (Group A), seven suffering from psychomotor seizure combined with generalized convulsion, from which complication they have been freed for over five years (Group B), and nine suffering from psychomotor seizure combined with continued generalized convulsion (Group C). These patients were over 10 years old at onset and have been followed up for 10 to 19 years at the Department of Neuropsychiatry of the Kansai Medical University Hospital. The seizure components in the long-term courses of these three groups were classified as they are under the heading of “partial seizures with complex symptomatology” in the “Clinical and Electroencephalographid Classification of Epileptic Seizures” proposed by the International League Against Epilepsy.

• 1 The nature of the seizures is monotonous and little varied in Group A and quite varied in Groups B and C. It seems to be a significant difference between isolated group and combined one regarding partial seizures with cognitive symptomatology. Partial seizures with dysmnesic disturbances seem to be closely related to the presence of generalized convulsion.
• 2 The contents of forced thinking tend to remain the same from one seizure to another in the isolated group, while they differ considerably in the combined one. In the strict sense, forced thinking differs from obsessive idea and is rather similar to autochthonous idea.
• 3 Episodic moodiness during seizure intervals is more clearly seen in the isolated group than in the combined one, but this tendency is also obvious in Group B.
• 4 Episodic psychotic state appears much more frequently in the combined groups than in the isolated one, and it appears on an average of 14 years of the course.
• 5 Deterioration in basic EEG rhythm is seen in a majority of cases, most of which belong to the combined group. Change in basic EEG rhythm is not seen in patients freed from generalized convulsion particularly in whom it has disappeared early.
• 6 In long-term clinical courses lasting more than 10 years, the disappearance of generalized convulsion leads to the disappearance or diminution of automatisms, but it also leads to the appearance or augmentation of autonomic seizure and affective seizure. Forced thinking tends to be perceptive and to develop to hallucinatory delusional experience through its repeated appearance over a long period of time.
• 7 As to outcome, all the isolated psychomotor cases improved, while half the combined cases showed no change or became worse. The prognosis for cases with continuous generalized convulsion is bad.

These findings suggest that many of the symptoms in patients with psychomotor seizures are closely connected with the presence of generalized convulsion.     

Chronic epilepsy with complex partial seizures is not always medically intractable--a long-term observational study

OBJECTIVE: To study the prognosis of patients with complex partial seizures (CPS) with or without simple partial (SPS) and secondarily generalized tonic-clonic seizures (GTCS) and to analyze the factors related to the degree of medical responsiveness. MATERIAL AND METHODS: A total of 266 adult patients with CPS were included in a hospital based observational survey with a follow-up of 2 to 25 years. Clinical characteristics, seizure frequency, electroencephalography (EEG), cerebral computed tomography (CCT) and magnetic resonance imaging (MRI) findings were analyzed. Patients were categorized according to their degree of medical responsiveness into one of three groups: seizure free, improved control (>50% seizure reduction) and poor control. RESULTS: Mean age at follow-up was 44.7 years (SD 14.7, range 19-93). Mean age at seizure onset was 18.1 years (SD 14.7, median 15, range 1-79). Complete seizure control was achieved in 40%, improved seizure control in 36% and poor seizure control in 24%. Patients entered remission after a mean period of 15.7 years (SD 12.6, median 13, range 1-54) of active epilepsy. A third of all seizure-free patients were still in remission 6.1 years (SD 5.3, median 3.5, range 1-18) after discontinuation of antiepileptic drugs (AED). Patients with poor seizure control had a significantly younger age at onset (P<0.01), a higher initial seizure frequency (more than 3 per month) (P<0.01), abnormal neurological examination (P<0.01), and were more often mentally handicapped (P<0.01). Multiple logistic regression analysis revealed a high initial seizure frequency, mental handicap and an abnormal neurological examination as independent risk factors for poor seizure control. A positive family history, a history of febrile convulsions and/or psychosis, an abnormal EEG or MRI was not predictive of poor outcome. CONCLUSIONS: Not all patients with CPS were medically intractable. Seizure remission can be achieved after a long time of active epilepsy. Poor seizure control was associated with a high initial seizure frequency, mental handicap and abnormal neurological examination.