Felix Rosenow

Semiological seizure classification

Summary: We propose an epileptic seizure classification based exclusively on ictal semiology. In this semiological seizure classification (SSC), seizures are classified as follows:

Histopathological Correlates of Epileptogenicity as Expressed by Electrocorticographic Spiking and Seizure Frequency

Summary: Purpose: To study the correlation between histopathology and epileptogenicity, as measured by seizure frequency and electrocorticography (EcoG), in patients with cortical dysplasia (CD) as compared with control patients with gangliogliomas or gliomas.

Sleep apnoea in treated acromegaly: relative frequency and predisposing factors

OBJECTIVESu2003Sleep apnoea is common in active acromegaly. It is associated with increased morbidity and mortality but can be treated effectively. The objective of this study was to determine the largely unknown relative frequency of, and the predictive factors for, sleep apnoea in treated acromegalic patients.

Sleep apnoea in endocrine diseases.

The pertinent literature on the prevalence, clinical manifestations and pathogenic mechanisms of sleep apnoea (SA) in endocrine diseases, namely acromegaly, Cushing syndrome, hypothyroidism and diabetes mellitus was reviewed. An increased prevalence is well documented in patients with active and treated acromegaly. While most authors report peripheral obstruction, due to hypertrophy of tongue and pharyngeal tissues, to be the cause of SA in acromegaly, some findings argue for a role of hormone‐induced changes of central respiratory control. SA is also more common in hypothyroidism, especially when myxedema is present. The associated edema and myopathy appear to be of pathogenic importance. Thyroxin substitution is frequently effective for the treatment of SA but nCPAP can be necessary initially and in some patients even after remission of clinical signs of hypothyroidism. In Cushing disease and syndrome, parapharyngeal fat accumulation can cause SA, but no epidemiological information is available. In non insulin dependent diabetes (NIDDM), obesity is the common risk factor for both, nocturnal hypoxia and insulin resistance. In IDDM, the development of autonomic neuropathy may predispose to SA. Where treatment of the underlying endocrine disease is unable cure the associated SA, nCPAP is usually the treatment of first choice. More prospective studies are clearly needed to establish prevalences and resolve the controversies regarding pathogenesis.

Staring spells in children: Descriptive features distinguishing epileptic and nonepileptic events☆☆☆★

OBJECTIVEnTo identify questions sensitive and specific for staring spells of epileptic (absence seizures [AS]) or nonepileptic etiology to increase the yield of history taking.nnnSTUDY DESIGNnA questionnaire was completed by parents of 40 children who presented with staring spells. Results from 17 children with AS and 23 with nonepileptic staring (NES) were compared.nnnRESULTSnFeatures with moderate sensitivity (43% to 56%) but high specificity (87% to 88%) for NES included preserved responsiveness to touch, lack of interruption of playing, and initial identification by a teacher or health professional. These features were more frequent in NES than in AS (P = .013, .016, .030). Body rocking occurred only in NES, but sensitivity was low (13%). Features with high specificity (91% to 100%) for AS included limb twitches, upward eye movements, and urinary incontinence; but sensitivities were low (13% to 35%).nnnCONCLUSIONnIn children with normal interictal electroencephalography findings and without neurologic disease, staring spells are most likely nonepileptic when parents report preserved responsiveness to touch, body rocking, or initial identification by a teacher or health professional without limb twitches, upward eye movements, interruption of play, or urinary incontinence. In these cases a diagnosis of NES may be confidently applied, with confirmation based on long-term follow-up.

Focal temporal burst-suppression during Wada-test correlated with speech arrest

This patient underwent a repeat Wada test after placement of subdural grid electrodes for chronic electrocorticographic recording (ECoG). Focal burst suppression pattern was recorded by the left temporal but not the suprasylvian subdural electrodes after intracarotid injection of 100 mg of sodium-amobarbital. Speech arrest continued until the recorded became record became continuous. In contrast to the prolonged hemispheric slowing by surface electroencephalogram (EEG), focal burst suppression on ECoG can be related tightly related with cortical dysfunction induced by intracarotid amobarbital injection.

Schilddrüse, Nebenschilddrüse und Epilepsie

There is clinical evidence that endogenous (hyperthyroidism) as well as exogenous thyroxin intoxications can provoke seizures or increase seizure frequency and intensity. The latter was reported particularly for juvenile myoclonic epilepsy (JME). Hypothyroidism, on the other hand, is associated with a decreased incidence of febrile seizures. Hashimoto-encephalopathy occurs in patients with Hashimoto- thyroiditis and is a usually steroid responsive, at times spontaneously remitting, encephalopathy. Epileptic seizures occurring in 50–60%, myoclonus psychotic or confusional states and changes in the level of consciousness are frequent neuro-psychiatric symptoms. Hypoparathyroidism, usually of iatrogenic etiology, frequently presents with tetany occurring within days or weeks of the operation. However, epileptic seizures were reported in 25–30% of the cases and can emerge months to years after the operation.ZusammenfassungKlinische Evidenz belegt, dass endogene (Hyperthyreose) aber auch exogene Thyroxinintoxikationen anfallsauslösend oder -verstärkend wirken. Letzteres wurde vor allem bei der juvenilen myoklonischen Epilepsie berichtet. Umgekehrt gibt es Hinweise, dass die Inzidenz von Fieberkrämpfen bei Hypothyreose vermindert ist. Die Hashimoto-Enzephalopathie ist eine mit der gleichnamigen Autoimmunthyreoiditis assoziierte akute wiederkehrende steroid-responsive, z.T. spontan remittierende Enzephalopathie, bei der epileptische Anfälle (in 50–60%), Verwirrtheit, Psychose, Störungen der Vigilanz und Myoklonien beschrieben sind. Beim meist iatrogenen seltener idopathischen Hypoparathyreoidismus können neben der meist bald nach der Operation symptomatischen Tetanie in 25–30% der Fälle, oft mit deutlich längerer Latenz, auch epileptische Anfälle auftreten.

Epilepsie bei rechtshemisphärischer dualer Pathologie

The presence of two possible epileptogenic lesions in one hemisphere presents a specific challenge in the presurgical localisation of the epileptogenic zone. Illustrated by such a case we discuss the importance of seizures semiology, ictal and interictal EEG and the predictors for the occurrence of seizures in patients with cerebral cavernomas.ZusammenfassungDas Vorliegen zweier, potentiell epileptogener Läsionen in einer Hemisphäre stellt eine besondere Herausforderung für die prächirurgische Lokalisationsdiagnostik dar. Anhand eines solchen Falles sollen hier die Bedeutung der Anfallssemiologie, des iktalen und interiktalen EEGs, sowie die Prädiktoren für das Auftreten epileptischer Anfälle bei Patienten mit zerebralen Kavernomen diskutiert werden.

Late-Onset Myoclonic Seizures in Down's Syndrome

Publisher Summary This chapter highlights the condition of a man diagnosed with late-onset myoclonic epilepsy in Downs syndrome. Late-onset epilepsy in elderly, demented Downs syndrome patients is characterized by myoclonus, occasional generalized tonic–clonic seizures or generalized myoclonic–tonic seizures, generalized epileptiform discharges on the EEG, and slow progression. Approximately 3 years before admission, myoclonic jerks had started. These usually occurred in the morning and could be improved by administration of valproate (1800 mg/day). The dose was reduced because of daytime somnolence, and this reduction coincided with the occurrence of the first generalized myoclonic–tonic seizure, as described by a witness. Apart from increased muscle tone and gait disturbance, physical examination did not reveal any significant neurological abnormalities. A mini-mental state examination yielded a score of 1 point. Computed tomographies scan revealed cortical atrophy; magnetic resonance imaging was not possible because of noncompliance on the part of the patient. The clinical diagnosis of Downs syndrome was confirmed by cytogenetical analysis. On a low dose of valproate (900 mg/day), the patients generalized myoclonic–tonic seizures were finally controlled but his myoclonic jerks increased over the subsequent months. Therefore, topiramate (100 mg/day) was introduced, which led to significant and sustained improvement. Epilepsy in Downs syndrome that is characterized by seizure onset after the fourth decade may comprise myoclonic jerks and occasional generalized tonic–clonic seizures associated with progressive dementia.