Swelling of the third nerve in a child with transient oculomotor paresis: a possible cause of ophthalmoplegic migraine.

Transient oculomotor nerve palsy is rarely observed in childhood and mostly is recognized to be a migraine equivalent. We report an 8-year old girl who presented with recurrent attacks of transient oculomotor paresis. The clinical profile of the patient suggested transient dysfunction in the context of an ophthalmoplegic migraine. A CT scan was normal, but MRI demonstrated a swollen right oculomotor nerve.

[Pseudoarthrosis in neurofibromatosis type 1]. / Pseudarthrose bei Neurofibromatose Typ 1.

Neurofibromatosis type 1 (NF1) is the most common neurocutaneous disease. The clinical manifestations are diverse. Some of the skeletal changes are most relevant to the patient. We report on 9 patients with NF1 who presented with typical pseudarthrosis. In 8 of these children the lower extremity was involved. In 2 cases lesions of both tibia and fibula were found, in one case even over long segments with a fully instable leg. One child had a complete pseudarthrosis of the radius and ulna. This report analyses the bony changes, the operations performed and the possible technical improvements to be made. The present study as well as other recent studies suggest that bony lesions should be operated early using microsurgical methods.

Rotatory seizures in a patient with tuberous sclerosis.

We present an 11-year-old girl with tuberous sclerosis who developed seizures characterized by circling behavior. Rotatory seizures are uncommon and occur mainly secondary to a focal pathology. Our patient had a right temporal epileptic focus, confirmed by magnetic resonance imaging (MRI) to be a subcortical lesion in the right temporal region. This case is probably the first reported case of tuberous sclerosis associated with rotatory seizures with an ictal EEG.

[Recommendations for blood studies and clinical monitoring in early detection of valproate-associated liver failure. Results of a consensus conferences 9 May - 11 May 1997 in Berlin]. / Empfehlungen zu Blutuntersuchungen und klinischer Uberwachung zur Früherkennung des Valproat-assoziierten Leberversagens. Ergebnisse einer Konsensus-Konferenz vom 9.5.-11.5.1997 in Berlin.

Valproate is a frequently used antiepileptic drug. It is associated with rare but serious adverse effects like liver failure. The first symptom is impairment of the patient's well being. Isolated changes of standard laboratory liver parameters are not reliable early indicators. Thus, according to the knowledge of today, prophylactic blood screening cannot predict complications. On the contrary, clinical symptoms are the most relevant indicators of impending complications, eventually supported by laboratory findings. An abrupt withdrawal of valproate and administering carnitin in parallel can interrupt the otherwise fatal course of the complication and induce a subsequent recovery. At a Consensus Conference the current knowledge about early detection and therapy of the valproate-induced serious hepatotoxicity was discussed. The results regarding recommended laboratory screening, as well as diagnostic and therapeutic strategies are reported.

Concomitance of childhood absence and Rolandic epilepsy.

Five children (3 girls and 2 boys) who showed generalized synchronous 3/sec spike and wave complexes as well as centrotemporal spikes in the same EEG or in different EEGs are described. Among these five patients only 1 boy and 1 girl showed clinically both absence seizures and partial motor seizures with or without secondary generalization. One girl and 1 boy have only absences and the other girl only partial motor seizures. A concomitance of generalized synchronous 3/sec spike and wave discharges and centrotemporal spikes in the same patient is rarely found. The clinical manifestation of absences and partial motor seizures in the same patient is extremely rare.

Apnoeic attacks as an isolated manifestation of epileptic seizures in infants.

Apnoea as an isolated manifestation of seizures is well described in neonates but is only occasionally observed in infants. We present data from four infants, with apnoea as the sole manifestation of seizures, documented by polygraphic ictal electroencephalogram (EEGC) and video recording. The four infants, after normal pregnancy and delivery at term, showed the first apnoea at the age of 2-11 months. The interictal EEG was normal. The ictal EEG and video recording showed in all infants a focal rhythmic alpha or theta activity with or without generalization, which lasted 40-120 seconds. The apnoea appeared a few seconds after the beginning of rhythmic activity and the heart rate remained unchanged during the apnoea. At 2 years' follow-up, three children are seizure-free under anti-epileptic therapy with normal psychomotor development in two, and a slight delay in the third infant. The fourth child has partial seizures and is severely retarded.

Acute encephalitis in Swiss children: aetiology and outcome.

Since published data on the course and prognosis of encephalitis in Central Europe is limited, we retrospectively evaluated 104 children with either acute strict sense encephalitis (n = 80) or acute cerebellar ataxia (n = 24) treated at the Department of Pediatrics, University of Bern, Switzerland, between 1980 and 1991. Of the 80 patients with strict sense encephalitis, four (5%) died acutely and 28 (36%) of 78 followed up had sequelae - eight patients with severe, six with moderate and 14 with mild sequelae. Young age and seizures were shown to correlate with poor outcome. Among the 24 patients with acute cerebellar ataxia, there was no fatal outcome and none developed severe residua, but six had mild and one had moderate sequelae. Initial cerebrospinal fluid white cell count was significantly higher in these children with sequelae compared with those without any sequelae after acute cerebellar ataxia.

Prolonged epileptic apneas in a newborn: a case report with ictal EEG recording.

We report a case of repetitive and prolonged apneas, correlating with EEG-focal epileptiform discharges over the right temporal region, in a full-term newborn. After administration of phenobarbital the apneas completely ceased. The neurological examination, the psychomotor development, and the EEG at the age of 12 months were normal. The prolonged duration of the epileptic apneas, the ictal rhythmic delta wave activity, and the favorable outcome distinguish our patient from the majority of previously reported cases.

Cardiac arrhythmias mimicking primary neurological disorders: a difficult diagnostic situation.

Cardiac arrhythmias can present with the signs and symptoms of a seizure disorder. This potentially life-threatening underlying cause of non-febrile seizures should be recognized early, since successful specific treatment is possible. The purpose of this retrospective study was to examine common features in such patients. Over a period of 25 years, eight patients were initially treated for up to 5 years at our institution for a seizure disorder until dysrhythmia as the underlying cause of the seizures was disclosed. The main symptom was drop attacks coinciding with physical activity or emotional stress. Convulsions were only rarely observed. In five of the eight patients the underlying disorder was the long-QT-syndrome (Romano-Ward syndrome). In one patient intermittent complete atrioventricular block was found, another patient showed ventricular tachydysrhythmia of unknown etiology and the last patient suffered from hypertrophic cardiomyopathy.

[Sleep disorders in childhood]. / Schlafstörungen im Kindesalter.

Sleep disorders in childhood are frequent and usually harmless. They rarely point to a serious disease. Difficulty in falling asleep and nightly awakenings are age-dependent and transitory. While the largely harmless somnambulism and pavor nocturnus occur in the deep sleep of the first third of the night, the anxiety dreams of REM sleep appear preferentially in the second half of the night. Other disorders such as nocturnal enuresis and talking in sleep may occur during the whole night. It is very important to inform the parents because this helps to counter fears and false expectations. Consistent sleep hygiene needs to be developed with avoidance of irregular sleep rhythm and an unrestful sleep environment. Sometimes it is necessary to learn new behaviour patterns with the child. Only exceptionally is drug therapy indicated. However, nocturnal breathing disorders and nocturnal epilepsy do have a pathological significance and need specific therapy. In order to clarify the reasons for sleep disorders, it is necessary to keep a sleep diary, to undertake specific examinations (e.g. psychological, pneumological, neurological, urological, otorhinolaryngological and possibly using diagnostic equipment such as video-polysomnography).

Factors predicting the risk of relapse after antiepileptic drug discontinuation in children with partial seizures.

The purpose of this study is to identify possible factors which could influence the seizure recurrence after anti-epileptic drug (AED) withdrawal in children with partial epilepsy. AED was discontinued in 82 children who had been free of partial epileptic seizures for 2.0-11.0 years (mean 4.7 years). Twenty-four patients (29.3%) had a relapse from a few days to 6.1 years (mean 1.2 years) after AED discontinuation. Significantly more common in children who relapsed were: younger age at beginning of AED withdrawal, occurrence of complicated febrile convulsions (5/24 vs 1/58, P < 0.01), abnormal neurological examination (8/24 vs 8/58, P < 0.05), delayed psychomotor development (7/24 vs 7/58, P < 0.05), focal slowing (6/24 vs 3/58, P < 0.01) and focal epilepti-form discharges (7/24 vs 6/58, P < 0.05) in the last EEG before AED discontinuation. Between the two groups no statistical significant differences concerning the age at onset of seizures, the duration at AED therapy after the last seizure, the familial occurrence of epilepsy and background EEG abnormalities in the last EEG before AED discontinuation were found. On the basis of EEG, occurrence of febrile convulsions, and neurological and developmental examination it may be possible to predict which children have the best chance to remain free of recurrence after AED discontinuation.

[Clinical results with sodium valproate in childhood and in adolescence]. / Klinische Erfahrungen mit Natriumvalproat im Kindesalter und in der Adoleszenz.

This is a review of own experiences and from the literature concerning efficacy and side effects of valproate in the treatment of generalized and partial epilepsies of childhood and adolescence.

[Neuropediatric emergencies]. / Neuropädiatrische Notfälle.

Febrile convulsions, afebrile seizures and headache are common acute problems in child neurology. The first steps of a practical approach by physicians in recognizing and treating these neurologic diseases are discussed.

Seizure types and epileptic syndromes.

The main epileptic syndromes occurring during brain maturation, i.e. during the neonatal period, infancy, childhood and adolescence, are reviewed. The concept of epileptic syndromes is of pragmatic significance and helps the physician to select appropriate modes of investigation, to predict the outcome and to choose the treatment of first choice. A delineation of epilepsies based on physiopathologic mechanisms and on etiology remains the ultimate goal.