Solitary epileptic seizure--the risk of recurrence.

Patients experiencing solitary unprovoked epileptic seizure have different risks of recurrence. The possible risk factors include in particular: structural cerebral lesion and its cause, history of febrile seizures, family history of epilepsy, the type of seizure, epileptiform EEG discharges and the problem of initiation or (or not initiation) of antiepileptic treatment after the first paroxysm. The factors shown above were evaluated in a group of 30 patients with solitary unprovoked epileptic seizure. Regarding recurrence of epileptic seizure, the only significant factor appeared to be initiation of treatment after the first unprovoked paroxysm (p<0.001). We observed a 30% and 33.33% risk of recurrence following the initial epileptic seizure in patients after the first unprovoked seizure in less than 1 and 3 years, respectively.

Metabolic stroke in three years old boy as the consequence of metabolic derangement. A case report of recidiving Reye's-like syndrome.

Three years old boy with developmental renal dysplasia was hit as newborn child by attack of cerebral edema with metabolic disturbances (hypoglycemia, hypophosphatemia, ketoacidosis and with hypocoagulation state) and was classified as child at risk in the pediatric evidence. In the third year of the age he went through nephrectomy and after the operation, the similar metabolic disturbances occurred (hypoglycemia, ketoacidosis, derangement of the metabolic situation). Cerebral edema and the metabolic stroke developed. Reye's-like syndrome was considered and serious functional disturbances of basal ganglia and brain-stem structure were observed.

Familial occurrence of adrenocortical insufficiency in two brothers with Allgrove syndrome. A case report of 4A (Allgrove) syndrome with epilepsy and a new AAAS gene mutation.

Allgrove syndrome is a rare autosomal recessive disease with achalasia, alacrima, adrenocortical insufficiency, autonomic neuropathy and other neurological disturbances. A case of two brothers with Addison s disease from early childhood is presented. The younger brother with Addison disease died at the age of 5. The older brother was treated for adrenocortical insufficiency from the age 3, and then treated for achalasia and epilepsy from the age of 5. The patient is currently 26 years old and suffers from achalasia and adrenocortical insufficiency. He also suffers from alacrima, autonomic neuropathy, epilepsy and other damages of the central and peripheral nervous system. The clinical picture is typical for Allgrove or 4A syndrome, and the diagnosis was confirmed by means of molecular analysis of a new AAAS gene mutation.

The quest of cavum septi pellucidi: obscure chance event discovery or the result of some encoded disturbance? Developmental cerebral dysplasias, cavum septi pellucidi and epilepsy: clinical, MRI and electrophysiological study.

OBJECTIVES: Developmental cerebral dysplasias are frequent causes of epilepsy. The early stage of gestation, mainly the period of neural crest separation and neuroblast migration (disturbance of midline structures, heterotopias, cortical dysplasias and disturbance of the ventricular and vascular formation), may be considered as a cause of serious cerebral dysplasia. The aim of the study was focused on frequent simultaneous occurrences of epileptic seizures and the defect or abnormality of the ventricular system - cavum septi pellucidi (CSP). MATERIAL AND METHOD: In our study the clinical symptoms, EEG and somatosensory evoked potentials (SEP's) following median nerve stimulation and MRI pictures in the group of patients with CSP (n= 35), were analyzed. In the SEP analysis, a control group of normal, healthy volunteers (n = 40) and a group of age matched patients with epileptic seizures of different origin, without structural lesions evident on MRI (n=21), were used. RESULTS: Analysis of the patient population with CSP (CSP was confirmed by MRI) showed that approximately in 2/3 cases, different types of cranio-cerebral dysplasias were evident on MRI. More than 2/3 of the patients with CSP showed epilepsy and an abnormal EEG record, however, focal EEG changes were seen more frequently in the group of patients with epilepsy without CSP, than in patients with CSP. The SEP's in patients with CSP showed a statistically significant prolongation of latency of thalamic P15 waves, however these changes were not present in the group of patients with epilepsy of a different origin. CONCLUSIONS: In a group of patients with CSP, dysplastic MRI changes, together with the prolongation of thalamic wave latencies according SEP, were examined. These clinical symptoms may be considered the result of disturbances of early gestation and of lesions of midline structures. CSP became an interesting model opportunity for us, and allowed for the clinical, MRI and electrophysiological examination of developmental cerebral dysplasias. We believe that there is an important role for septal and diencephalic midline structures in cerebral electrogenesis, and possibly in the origin of epileptic seizures too.

Spinal cord lesions in diabetes mellitus. Somatosensory and motor evoked potentials and spinal conduction time in diabetes mellitus.

OBJECTIVES: Diabetic neuropathy and autonomic nervous system neuropathy are recognized as the most common clinical pictures of nervous system disorders caused by diabetes mellitus (DM). Damage to the brain and the spinal cord is rare. The aim of this work is to show the importance of somatosensory and motor evoked potentials (SEP and MEP) for the early diagnosis of nervous system damage related to diabetes mellitus. MATERIAL AND METHODS: We examined spinal and cortical somatosensory evoked potentials (SEP) after median and fibular nerve stimulation in diabetics and control subjects. We measured the latencies of individual wave deflections and peripheral and central conduction time (PCT and CCT) of spinal and cortical SEP. Similarly, transcranial magnetic stimulation was used for measuring the central and peripheral conduction time (CCT and PCT) in a group of type 1 diabetics and a control group of volunteers. RESULTS: The examination SEP and MEP proved and confirmed the prolongation not only of peripheral conduction time, but also of the central conduction time - especially in spinal cord structures. An assumption that spinal cord changes are connected with the decreased number of myelinated fibers able to conduct the impulses from periphery and brain cortex, respectively, has to be accepted. CONCLUSIONS: The results suggest that the use of somatosensory and motor evoked potentials (SEP and MEP) examination and conduction times measurement has significance in the confirmation of unapparent lesions of the spinal cord in diabetics of both types.

Familial occurrence of myoclonic epilepsy syndrome and acute intermittent porphyria.

OBJECTIVES: Myoclonic epilepsy (ME) syndrome is not rare in north-eastern Europe; it is also seen in various forms. Familial occurrence of ME syndrome and acute intermittent porphyria (AIP) was observed in three siblings. The following report was aimed the differentiation between co-morbidity of two different disorders or presence the epileptic seizures within the clinical picture of latent AIP. MATERIAL AND METHODS: A case report of three siblings who suffered from seizures, myoclonias, ataxia and minor psychological changes since the age of 8 and 9 years is described in the following report. RESULTS: The clinical picture most resembled that of "Baltic myoclonus" (dentate-rubral degeneration or dyssynergia cerebellaris myoclonica -- Ramsay-Hunt syndrome) with epilepsy and/or a benign form of progressive myoclonic epilepsy (PME). The possibility of juvenile myoclonic epilepsy (JME) and other aetiological factors, as less probable causes of ME syndrome, were considered. After 15 years of the treatment by anti-epileptic drugs in all three siblings, AIP was discovered. CONCLUSION: Our interest lies in the differentiation of co-morbidity of two different disorders or presence of epileptic seizures as the clinical picture of latent AIP. We propose that the AIP attacks were caused by long-term administration of anti-epileptic drugs. At the same time we suggest it is a coincidence that the two independent genetic abnormalities coexist in the subjects (benign form of degenerative cerebral disease and AIP).

Pathological somatosensory evoked potentials in patients with hypertension: a contribution to the study of the pathophysiology of juvenile essential hypertension.

BACKGROUND: The role of factors influencing the cerebral regulation of blood pressure must be considered in the etiopathogenesis of essential hypertension The aim of our work was to explore the possibilities for differentiating certain clinical, functional and electrophysiological signs in young and adult hypertensives. This could help us to determine whether centrally active antihypertensive drugs may be more suitable than others. MATERIAL/METHODS: We examined somatosensory evoked potentials (latencies of wave deflections and amplitudes) in two groups of patients with essential hypertension. The first group consisted of 25 hypertensive patients aged 20 to 30 years with onset before age 20 (n=25). The second group consisted of 40 hypertensive patients aged 40 to 60 years with onset after the age of 30. The results were compared with those obtained from age-correlated healthy controls. RESULTS: The most interesting changes were observed in the amplitudes of somatosensory evoked potentials in the group of juvenile patients with hypertension. The decrease in the amplitude of all wave deflections was very compact, consistent, and statistically significant even after repeated examinations. This finding raises the possibility of influence or damage to the peripheral afferentation of signals in juvenile patients, with hypertension as an expression of the reactions of brainstem structures. CONCLUSIONS: Our results suggest that afferent impulses and the reactivity of brainstem structures may play a role in the etiopathogenesis of hypertension.