Merosin-negative congenital muscular dystrophy, occipital epilepsy with periodic spasms and focal cortical dysplasia. Report of three Italian cases in two families

We report clinical, EEG and neuroimaging findings of three patients in two Italian families with merosin-negative congenital muscular dystrophy (CMD), drug-resistant occipital epilepsy, diffuse persistent cerebral white matter changes and focal cortical dysplasia. Clinical and epilepsy histories, EEG and neuroimaging findings were very similar in all patients. Seizures started in childhood and mainly consisted of periodic spasms, a particular type of partial seizure characterized by clusters of epileptic spasms. The motor expression of the spasms was very mild so that they had been frequently missed or misinterpreted as non-convulsive generalized absence seizures. Interictal EEG showed occipital spike-waves and bilateral synchronous slow spike-wave discharges. Ictal EEG showed prolonged periodic sequences of slow waves with associated fast rhythm complexes, characteristic of periodic spasms. Two patients had normal intelligence, one patient presented moderate mental retardation. Focal cortical dysplasia in the posterior areas of the brain, in addition to marked diffuse white matter alterations, was detected in the magnetic resonance images of all patients. Findings in these patients indicate that in merosin-negative CMD brain involvement can include cortical dysplasia, in addition to white matter changes. In such cases the brain damage can lead to a childhood-onset localization-related symptomatic occipital epilepsy. Epileptic seizures and cortical dysplasia can be, however, difficult to detect in CMD. The clinical semiology of epileptic seizures may in fact be modified because of muscular weakness. This implies that epilepsy may be misdiagnosed or even missed and EEG-polymyographic recordings may be necessary to identify it. Similarly, cortical dysplasia may be very localized and visible by neuroimaging only if it is carefully investigated on the basis of epileptological and EEG-polymyographic findings.     

Repair of traumatic orbital wall defects with nasal septal cartilage: report of five cases

PURPOSE: Arachnoid cysts are sometimes encountered in MRIs performed for a variety of reasons. In patients with epilepsy, particularly those with refractory epilepsy, arachnoid cysts are often assumed to be related to their seizure focus. We conducted a study to investigate this putative relationship. METHODS: A retrospective study on the incidence of arachnoid cysts was performed in patients seen in our Epilepsy Clinic who had CT or MRI scans, interictal EEGs or ictal EEGS. Locations of seizure foci in these patients were defined from clinical and electrophysiologic data. RESULTS: Seventeen of 867 patients had arachnoid cysts. Twelve patients had temporal lobe cysts and only 3 of them had temporal lobe seizures. Four patients had frontal lobe cysts and only 1 had frontal lobe seizures ipsilateral to the cyst. One patient had a cerebello-pontine angle cyst and frontal lobe seizures. Thus, clinical manifestations of seizures and EEG findings (interictal and/or ictal) indicated that the seizure focus was adjacent to the cysts in only 4 patients (23.5%). CONCLUSIONS: Our findings suggest that arachnoid cysts are often an incidental finding in patients with epilepsy and do not necessarily reflect the location of the seizure focus.     

The effectiveness of computer-assisted cognitive rehabilitation for persons with traumatic brain injury

Electroencephalographic (EEG) findings in syncope are reviewed. There are four major categories of syncope: neurally mediated (neurocardiogenic), neurologic, decreased cardiac output, and orthostatic hypotension. However, regardless of cause, whether the syncope is due to a vasovagal effect, a cardiac arrhythmia, an epileptic seizure, or hypotension, EEG findings are similar and reflect cerebral hypoperfusion. Initially there may be a slowing of background rhythms. This is followed by high amplitude delta activity, maximal anteriorly. If the hypoperfusion persists there is subsequent flattening of the EEG. The EEG returns to normal in the reverse sequence. In cases with severe and prolonged ischemia, convulsive syncope may occur at the time of the EEG flattening. Although not an epileptic phenomena, clinically this is often mistaken for epilepsy. Conversely, epileptic disorders, such as the ictal bradycardia syndrome, may occasionally mimic syncope. Therefore, in patients in whom EEGs are performed for the evaluation of an episode of loss of consciousness, simultaneous ECG should be used.     

The p21Rac/Cdc42-activated kinases (PAKs)

PURPOSE: Purely subjective events, identified as "seizures" by patients but not accompanied by any clinical or EEG changes, commonly occur during evaluation of patients for intractable epilepsy. We wished to determine the significance of such events. METHODS: We analyzed the frequency of subjective episodes in 379 adults who underwent EEG video monitoring as part of their evaluation for intractable epilepsy and the relationship of these experiences to epileptic and psychogenic nonepileptic (NES) seizures. We examined the results of tests of mental ability and of emotional, psychosocial, and quality of life (QOL) adjustment to determine if individuals with subjective events alone could be distinguished from those with epileptic seizures only, NES only, or no events, by these measures. RESULTS: We noted subjective events in 39% (147 of 379) of the patients. Of patients with subjective episodes, 52% also had epileptic seizures, 7% had NES, 1% had both epileptic seizures and NES, and 40% had subjective events alone. As a group, patients with subjective events alone were slightly more intelligent and less impaired neuropsychologically than people in the other groups, but they performed more poorly on several important measures of adjustment. CONCLUSIONS: The identification of persons with purely subjective episodes apart from all other episodes is important both phenomenologically and from a practical viewpoint.     

Incidence of photosensitive epilepsy: a prospective national study

We undertook a prospective nationwide study to determine the incidence of photosensitive epilepsy (PE). Virtually all EEG departments in Great Britain (providing approximately 90% coverage of all EEGs performed on people with newly diagnosed seizures) screened cases referred to them over a 3 month period and identified all new cases of epilepsy (defined as one or more recognised seizures) whose first EEG showed a photoparoxysmal response (PPR) on intermittent photic stimulation (IPS). 191 cases were identified, 143 of whom had type 4 PPRs (generalised spike and wave on IPS) on their first EEG. The annual incidence of cases of epilepsy with type 4 PPRs on their first EEG was conservatively estimated to be 1.1 per 100,000, representing approximately 2% of all new cases of epilepsy. When restricted to the age range 7-19 years, the annual incidence rose to 5.7 per 100,000-approximately 10% of all new cases of epilepsy presenting in this age range. To ascertain if there was a significant seasonal variation in PE, 5 EEG departments (which together contributed 15% of cases in the first study period) were visited during a second 3 month study period to identify all new cases of epilepsy with type 4 PPRs on their first EEG. No significant seasonal variation in incidence between summer and winter was found.     

Reading-induced epilepsy: three new cases

INTRODUCTION: Primary epilepsy of reading is a rare syndrome in which patients present with mandibular myoclonia when reading texts. Seizures are also frequently provoked by other stimuli. Occasionally they may be followed by generalized tonic-clonic seizures. CLINICAL CASES: We present the clinical features and electroencephalographs of three patients with epilepsy of reading, one of them also had seizures when playing chess and after doing arithmetic and the third after reading music. Treatment with valproic acid and clonazepam completely controlled the seizures in all three patients. In two cases we saw epileptiform activity on EEG whilst they were reading, although the basal EEG was normal. CONCLUSIONS: We believe that epilepsy of reading is under-diagnosed and emphasize the importance of careful questioning of epileptic patients as to factors which may precipitate the seizures. In view of the social problems which may arise, early recognition of the syndrome is important for the patient.     

Reflex epilepsies: experience in Sri Lanka

Reflex epilepsy (RE) is characterised by seizures that are regularly elicited by some specific stimulus or event mediated by neural pathways. In a prospective study of 1287 epileptic patients seen at Peradeniya, 223 (17.3%) were found to have RE, eating being the commonest stimulus (191 patients, 85.7%). Photosensitive epilepsy (PSE) was relatively rare. Intermittent photic stimulation on 874 unselected epileptic patients produced a positive photoconvulsive response in 60 (6.9%). None had photosensitive seizures, but 3 had a higher frequency of seizures while watching television. Eating epilepsy (EE) had the highest prevalence at Peradeniya (148/1000 epileptic patients). This group was male predominant, and the onset of epilepsy in most cases was in the second decade. The majority experienced partial complex seizures. Repetitive and chronic stimulation of the amygdala during eating is suggested as the mechanism underlying EE. Twenty-one patients had seizures evoked by calculation, problem solving or spatial tasks. Juvenile myoclonic epilepsy was the commonest form of seizure disorder in them. Although PSE itself is rare, self-induced epilepsy (SIE) was common. There were 8 patients who self-induced seizures. The majority were photosensitive and they induced seizures by gazing at the sun and waving a hand in front of the eyes. In the management of REs, clobazam produced impressive results. As for possible seizure-inhibitory mechanisms, our studies on a "Sathi" mediator showed definite EEG changes during mediation. Can mediation increase the seizure-threshold and abort or prevent the propagation of the epileptic discharge? The answer, apart from its possible therapeutic applications, may provide insight into the mechanisms of seizure generation.     

Dynamic cerebral autoregulation during sevoflurane anesthesia: a comparison with isoflurane

OBJECTIVE: To study the incidence and pattern of epilepsy in patients with periventricular leukomalacia (PVLM) in two specialty clinic settings. BACKGROUND: Motor and cognitive deficit as well as epilepsy are common in patients with PVLM. With modern imaging techniques, PVLM is now easily recognized. METHODS: Epileptic seizures and syndromes as well as motor and cognitive deficits were correlated with MRI findings. Two patient populations were studied: Group A-children with cerebral palsy and PVLM presenting to a center for children with motor disability (n = 19); and Group B-epileptic patients with PVLM presenting to a tertiary epilepsy center (n = 12). A single patient with PVLM and epilepsy who underwent extensive investigations, including intracranial EEG telemetry, is reported. RESULTS: In Group A, 47% of patients had epilepsy (9/19). PVLM was found in 1.27% of patients investigated for epilepsy at a tertiary epilepsy center (12/942). The majority of patients in both groups had multiple seizure types, with complex partial seizures being most common. Of patients with seizures (Groups A and B), 85.7% had intractable epilepsy (18/21). Intracranial EEG in the illustrative case demonstrated a multifocal epileptic process with occipitotemporal predominance. CONCLUSIONS: PVLM was an uncommon underlying cause in patients presenting with epilepsy (Group A); however, patients presenting with motor disability and PVLM (Group B) had a high incidence of seizures. PVLM in epileptic patients is associated with multiple seizure types and medically refractory disease.     

Spectral EEG during short-term discontinuation of antiepileptic medication in partial epilepsy

The effect of short-term discontinuation of antiepileptic drugs (AEDs) on spectral analysis of EEG background activity (spectral EEG) was studied in patients undergoing preoperative evaluation for epilepsy surgery. We also wished to clarify whether AED discontinuation would provide lateralizing evidence in spectral EEGs of patients with temporal lobe epilepsy (TLE). Spectral EEGs were performed in 15 patients receiving their regular medication regimen and again after a 1-week period during AED withdrawal. A subgroup of 9 patients who had previously undergone temporal lobectomies (TLE group) was studied separately. In this group, we evaluated the effect of preceding seizures on spectral EEG derived from temporal neocortical areas. In all patients, spectral EEG changes were detected even after a short-term AED withdrawal. The total amount of absolute delta activity was reduced and occipital peak frequency and relative alpha activity were increased as compared with baseline values. In TLE patients with habitual seizures occurring < 20 h before the spectral EEG recording, lateralization was correctly identified by the greater amount of absolute delta activity ipsilateral to the epileptogenic focus. Epileptic seizures are accompanied by subtle focal slowing in spectral EEG for a much longer period than has been assumed. In addition, postictal spectral EEG over the temporal lobes may have lateralizing value. Further studies in larger numbers of patients are needed to evaluate the role of spectral EEG in the preoperative evaluation of patients for temporal lobe surgery.     

Seizures involving the supplementary sensorimotor area in children: a video-EEG analysis

Most reports of supplementary sensorimotor seizures have been of adults with medically refractory epilepsy. Typically, supplementary sensorimotor seizures have onset in childhood. We describe the electroclinical features in 12 children. Cases were selected from an EEG laboratory population in whom video-EEG was performed routinely on all children. Supplementary sensorimotor seizures were diagnosed when there was bilateral tonic posturing of the upper or lower extremities, preserved consciousness, and lack of postictal confusion. Sensory auras were reported by 8 of 9 children aged > 3 years who had daytime seizures. Speech arrest occurred in all patients in whom it could be assessed, and abnormal vocalization was observed in 7 children. Interictal EEGs were often normal (49% of recordings), and ictal EEG changes could be subtle. An etiology was demonstrated in only 2 children, and brain imaging studies were normal in the other 10 patients. Seizures were controlled with antiepileptic drugs (AEDs) in 6 of the 12 children. The clinical manifestations of supplementary sensorimotor seizures in children are similar to those reported in adults; misdiagnosis is common at all ages.     

Frontal lobe epilepsy in infancy: is there a benign partial frontal lobe epilepsy?

INTRODUCTION: Only the organic and cryptogenic forms of frontal lobe epilepsy have been admitted by the ILAE, but according to several reports in the literature, it probably exists also an idiopathic form, at present not well recognized. OBJECTIVE: To study the differences between the organic and the cryptogenic forms of frontal epilepsy in our patients, that might indicate the presence of idiopathic cases within the presumed cryptogenic group. MATERIAL AND METHODS: All patients diagnosed of frontal lobe epilepsy, EEG registered, at the neuropediatric clinic of our hospital during 1993-1996, were selected: 30 patients under 14 years of age that were classified as organic (10 cases) or cryptogenic (20 cases) according to CT or MRI findings. The Mann-Whitney test and the Fisher exact test were performed for statistical analysis. Five patients with peculiar neurocognitive symptoms, epilepsy-related, are described in detail. RESULTS: Significative differences between the organic and cryptogenic forms of frontal lobe epilepsy were not found except for the tendency of cryptogenic EEG foci to spread beyond the frontal lobe, to generalize and to be associated with foci of other localizations. Epileptic status, either convulsive or not convulsive, were a frequent complication in both groups. CONCLUSIONS: The identification of an idiopathic form of frontal lobe epilepsy is difficult by its low prevalence as compared to the cryptogenic and organic forms, by the tendency of frontal seizures to become epileptic status, which predicts an intractable epilepsy, and by the clinical characteristics essential to frontal discharges: heterogeneity, frequent impairment of consciousness and specially the neurocognitive semiology, including dementia, that occurs as a critic or paracritic phenomenon, and that sometimes may develop or be accentuated by antiepileptic medication.     

Involvement of insulin-like growth factors-I and -II and their receptors in medroxyprogesterone acetate-induced growth of mouse mammary adenocarcinomas

Data about psychiatric disorders associated with epilepsy as well as their risk factors are heterogeneous. The overall prevalence of psychiatric disturbances in epileptic patients can be estimated between 20 and 30 per cent. It is the highest in pharmocoresistant cases seen in specialized centers. Psychotic disorders, depression, and suicide are the three most common among interictal disturbances. Psychoses affect 2 to 9 per cent of patients and are more frequent in cases with aura or altered consciousness, such as in complex partial seizures and absences. They correlate positively with the multiplicity of seizures but often inversely with their frequency. Temporal lobe epilepsy is associated with schizo phrenic-like and paranoid types of psychosis, but frontal lobe epilepsy is also common. A putative association with predominant left or bilateral EEG abnormalities in cases with partial epilepsy remains to be confirmed, as well as the frequency of underlying structural lesions. Depressive disorders affect 20 to 60 per cent of patients. While their occurrence with partial complex seizures and left hemisphere foci is common, the role of temporal lobe involvement still appears controversial. Depression prevails in cases with seizures that occasionally, albeit rarely, secondarily generalize and correlates with the duration of the disease, intractable seizures, and polypharmacy. A genetic factor is likely to play a role. Suicides rates are increased, encountered in 0.2-0.5 per cent of patients and causing deaths in 3-7 per cent of them. The overall risk might be the highest during the first years after diagnosis of epilepsy, as well as in patients with temporal lobe foci, depression, or psychosis. Great variability and discordance in results show the major difficulties encountered in epidemiologic studies. Most of these problems relate to the classification of epileptic disorders as well as that of psychiatric disorders, the variability in the methods and measures which are used, and frequent bias in the selection of patients. We review here data about the frequency of major psychiatric disorders in epileptic patients or the frequency of epileptic disorders in psychiatric patients, and also possible risk factors related to the epileptic disease and its evolution.     

EEG findings in frontal lobe epilepsies

As a group, epilepsies of frontal lobe origin are thought to be poorly localized using surface EEG recordings. This finding may depend on the specific areas of frontal lobe from which the seizures originate or the pathologic substrate. We reviewed the presurgical surface EEGs of patients with frontal lobe epilepsy who underwent epilepsy surgery. The specific area of the frontal lobe where seizures originated was determined by 1) intracranial ictal EEG recordings, or 2) the presence of a structural lesion, identified by imaging studies in patients who achieved complete seizure control following surgery. We differentiated patients whose seizures began in the dorsolateral frontal convexity from those whose seizures began in the medial frontal region, and we correlated EEG findings in the interictal, postictal, and ictal states with seizure semiology, pathologic substrate, and surgical outcome. Four of nine patients had seizures originating in the dorsolateral frontal convexity and five had medial frontal onset seizures. Patients whose seizures originated from the dorsolateral convexity had focal interictal epileptiform abnormalities that localized to the region of seizure onset. Patients whose seizures began in the medial frontal region had either no interictal epileptiform abnormality or had multifocal epileptiform discharges. Patients whose seizures began in the dorsolateral convexity showed focal electrographic seizure activity that was localizing. This rhythmic fast activity did not appear to be substrate-specific. Patients whose seizure onset localized to the medial frontal region did not show focal electrographic seizure at clinical onset. We conclude that the scalp EEG recordings of frontal lobe epilepsies contain features that enable differentiation of seizures originating from two different regions of the frontal lobe.     

Histological change of keratinocyte in full-thickness skin autograft and its effect on hyperpigmentation of the graft

OBJECTIVES: To investigate the frequency of ECG abnormalities suggestive of myocardial ischaemia in patients with severe drug resistant epilepsy and without any indication of previous cardiac disease, assuming that these changes may be of significance for the group of epileptic patients with sudden unexpected death. MATERIAL AND METHODS: Twelve patients with medically intractable epilepsy were investigated with simultaneous long ECG and EEG recordings while attending either epilepsy surgery investigational procedures or the investigational programme for diagnostic purposes, and one while having an episode of status epilepticus. RESULTS: The ECG recording failed in 1 patient. This patient had chest pain and minor yet morphologically conspicuous changes in the ECG, suggestive of myocardial infarction. He died in heart arrest. Eight epilepsy patients had episodes of ST segment depression in the ECG, many of which coincided with video- and EEG documented epileptic seizures. Two patients experiencing simple partial seizures and 1 patient experiencing absence seizures had no ST segment depressions in the ECG. One patient had an episode of status epilepticus secondary to brain damage and no ST segment deviation was seen during the ECG recording which continued until 3 h before the patient died. CONCLUSION: Patients with severe drug resistant epilepsy have episodes of ST segment changes, some of which are closely related to epileptic seizures. Further studies are needed to confirm the present results and to investigate the nature of these changes and document the effect of prophylactic treatment with cardioactive drugs to reduce the risk of sudden death.     

EEG and clinical studies of the development of alumina cream epileptic focus in split-brain cats

Alumina cream epileptic focus was established in the right sensorimotor cortex in 20 split-brain cats (partial or complete). EEG and behavioral observations were made in a period ranging from 24 to 836 days. Four types of EEG changes after alumina cream injection were differentiated. These types could be related to the direct effects of brain damage and to development of epilepsy. Spikes and sharp waves and paroxysmal discharges (focal and multifocal) were observed in about 60% of the cats. Clinical seizures developed in about the same percentage of the animals. These values are below those reported for cats with intact interhemispheric commissures. Diphenylhydantoin (DPH) was given orally in a daily dose of up to 15 mg/kg body weight in 9 animals with developed epileptic EEG activity. Five of them had epileptic seizures. DPH was introduced not earlier than 1.5 months after intracortical alumina cream injection. The plasma level of DPH varied between 7-20 mug/ml. This dose produced chronic symptoms of intoxication. Neither EEG changes nor clinical seizures were entirely controlled by this drug. Additional doses of Relanium (diazepam), and phenobarbital were necessary to stop generalized seizures or status epilepticus.     

Epilepsy and the heart

The relations between epilepsy and heart are complex and expressed in two opposite sides. (1) Cardiac arrhythmias may provoke epileptic seizures but these seizures are, in this case, syncopal attacks. Nevertheless, in the past, these clinical features have been individualized as "cardiac epilepsy" or epilepsy in cardiacs. However, true epileptic seizures could be observed in the course of a syncopal attack and a syncope may complicate the issue of an epileptic seizure. (2) On the other hand, epileptic seizures may provoke severe cardiac arrhythmias. The incidence rate of sudden death in patients with epilepsy is estimated to be 1/1000 patients. The exact neural mechanisms in cardiac arrhythmias seizures could explain only some of the sudden unexpected deaths observed in epileptic patients. The role of antiepileptic drugs on cardiac conduction as well as the effects of seizures or status epilepticus on the myocardium are other enigmatic aspects of the relations between epilepsy and heart.     

The evolution of neurophysiological features in holoprosencephaly

The evolution of EEG, visual and auditory evoked responses (VER and AER) and sleep is described in three cases of semilobar holoprosencephaly. During the neonatal period, the waking EEG was characterized by almost continuous high amplitude rhythmic alpha-theta activity in case 1 and 2, which became discontinuous during quiet sleep. Moderate amplitude rhythmic alpha-theta waves were seen in case 3. This rhythmic alpha-theta activity gradually disappeared with increasing age, being replaced by non-specific slow dysrhythmia. In case 3, the subsequent EEGs were characterized by focal spikes at 4 months, multifocal spikes at 5 and 6 months, hypsarrhythmia at 8 months and bisynchronous diffuse sharp and slow wave discharges at 2 years and 7 months. Ictal EEGs were characterized by desynchronization and/or rapid synchronization, epileptic recruiting rhythm and postical high amplitude slow waves. Definite but mostly abnormal VERs or AERs were obtained in all three cases. In two cases, the evoked responses showed a progressive decrease in amplitude and VERs were abolished finally. No sleep cycle could be identified during the neonatal period probably because of frequent seizures. In two cases no circadian rhythm of sleep developed, although almost normal REM-NREM sleep cycle was present.     

Electroencephalographic findings in Tanzanian epileptic patients

Electroencephalogram (EEG) of 524 Tanzanian epileptic patients seen between 1985 and 1987 were reviewed. Over two thirds were young patients between the ages of five and thirty. Four hundred and fifty (86%) had abnormal records. Eighty nine per cent of abnormal records had focal abnormality and 11% had centrencephalic abnormality. Grand mal seizures did not imply centrencephalic abnormality, only 13% had such abnormality. Petit mal seizures are rare, over one third of these had temporal focal abnormality. Partial seizures were associated highly with focal abnormality. However, complex partial seizures did not imply temporal focal abnormality. Implications of EEG findings to correlation with epileptic seizures is discussed.     

Juvenile myoclonic epilepsy

CONCLUSION: We conclude that despite inevitable variability the clinical picture of JME is characteristic. It is easy to diagnose JME if one thinks of it while the history should be thoroughly analyzed. An EEG recording during sleep confirms the diagnosis. An early diagnosis of JME permits adequate prognosis of the subsequent course of epilepsy, and adequate therapy brings remission in most of the patients. If treatment starts following the large number of severe GTC seizures, the response to therapy is incomplete. The persistency of the illness throughout the life, the need for continuous medication and therapeutic unresponsiveness in cases with late diagnosis, do not justify the increasing misconception that JME is of benign nature. Diagnosis of JME is rare because of insufficient familiarily of physicians with the illness. BACKGROUND: Juvenile myoclonic epilepsy (JME) is an idiopathic generalized epileptic syndrome characterized with the combination of myoclonic, generalized tonic-clonic (GTC) and absence seizures that are readily provoked by sleep deprivation. PATIENTS: Forty-three patients, aged from 14 to 51 years, participated in a 5-year follow-up study. Diagnosis was made according to the criteria (Table 1) for diagnosis of JME set by Panayiotopoulos et al. (1994). Nineteen patients made their first contact with a neurologist at the Institute of Neurology and were diagnosed as JME, while the remaining 24 were referred to from other medical institutions with a diagnosis of therapy resistant to focal epilepsy. All patients underwent a somatic and neurological examination, "mini mental test," EEG in waking and CT scan of the brain. Some patients had EEG performed during sleep and some had MRI of the head. RESULTS: JME began between 9 and 26 (average 17) years. All patients had myoclonic seizures, 98% had GTC and 23% absence seizures. The first myoclonic seizure occurred between 9 and 24 years while the frst GTC seizure occurred between 10 and 32 years. Myoclonic seizures (83% of patients) and GTC seizures (70% of patients) occurred most often immediately after awaking. The most frequent provocative factors were insufficient sleep, alcohol abuse and tiredness. Epilepsy in the family was present in 39%, focal neurological deficiency in 9% and pathological findings on of CT and MRI in 7% of patients. Waking EEG was pathological in 77% of patients; it included generalized spike-wave discharges in 73%, multiple spike-wave complexes in 33% and focal discharges in 12% of patients, respectively. In all 26 patients tested, sleep EEG was pathological most often with multiple spike-wave complexes in 85% and 3-4 Hz spike-wave complexes in 57% of patients. The correct diagnosis of JME following a comprehensive examination was made in 24 (56%) patients after a delay of 1 to 35 years. In 24 patients with delayed diagnosis of JME the replacement of earlier medication with valproic acid (VPA) induced remission in 18 patients (75%) while 1 patient (4%) experienced a reduction in the number of seizures. Five patients (21%) did not respond to VPA medication: 2 due to a weak compliance, another 2 due to inefficient medication and 1 because of the preexistent malabsorption syndrome. In 19 patients (44%) with initial diagnosis of JME, VPA was introduced immediately upon diagnosis. Of them, 15 (79%) had excellent response to VPA, 1 refused therapy and for 3 patients there is no information. In 2 patients VPA was substituted due to side effects (hepatotoxicity and alopetia) with lamotrigine (low doses), which brought about decrease in frequency and mitigation in myoclonic seizures.     

Retinal vessel dilatation and elongation precedes diabetic macular oedema

Rolandic discharge (RD), noted in the electroencephalography (EEG) of patients with benign epilepsy in childhood with centrotemporal spikes (BECCT) has several unique features. One feature is that the amount or frequency of RDs does not correlate well with the incidence of seizures in BECCT although it is a key finding in the diagnosis of this epileptic syndrome. In this study, we examined the efficacy of antiepileptic drugs focusing on the disappearance of RDs in relationship with seizure control. Forty patients with BECCT who were not medically treated prior to this study were randomly sorted into three groups. Twenty patients were assigned for clonazepam (CZP) treatment, 10 patients for valproate (VPA) and the remaining 10 patients for carbamazepine (CBZ). Each drug was administered for 4 consecutive weeks. EEGs were recorded twice during the study, before and 4 weeks after the medication trial. The effects of each treatment on RDs were assessed. RDs disappeared in 15 of the 20 cases treated with CZP (75%) within 4 weeks while the same was observed in only one of the 10 cases treated with VPA (10%). CBZ failed to demonstrate any effect on RD. In the group treated with CZP, there were no differences in seizure incidence, seizure type and blood concentration of CZP between the patients whose RDs disappeared and those whose RDs remained.