Stroke-like Episodes in Inherited Neurometabolic Disorders.

Stroke-like episodes (SLEs) are significant clinical manifestations of metabolic disorders affecting the central nervous system. Morphological equivalents presented in neuroimaging procedures are described as stroke-like lesions (SLLs). It is crucial to distinguish SLEs from cerebral infarction or intracerebral hemorrhage, mainly due to the variety in management. Another significant issue to underline is the meaning of the main pathogenetic hypotheses in the development of SLEs. The diagnostic process is based on the patient's medical history, physical and neurological examination, neuroimaging techniques and laboratory and genetic testing. Implementation of treatment is generally symptomatic and includes L-arginine supplementation and adequate antiepileptic management. The main aim of the current review was to summarize the basic and actual knowledge about the occurrence of SLEs in various inherited neurometabolic disorders, discuss the possible pathomechanism of their development, underline the role of neuroimaging in the detection of SLLs and identification of the electroencephalographic patterns as well as histological abnormalities in inherited disorders of metabolism.

Gastrointestinal and Autonomic Symptoms-How to Improve the Diagnostic Process in Panayiotopoulos Syndrome?

One of the most common epileptic disorders in the pediatric population is Panayiotopoulos syndrome. Clinical manifestations of this idiopathic illness include predominantly autonomic symptoms and dysfunction of the cardiorespiratory system. Another feature constitutes prolonged seizures that usually occur at sleep. It is crucial to differentiate the aforementioned disease from other forms of epilepsy, especially occipital and structural epilepsy and non-epileptic disorders. The diagnostic process is based on medical history, clinical examination, neuroimaging and electroencephalography-though results of the latter may be unspecific. Patients with Panayiotopoulos syndrome (PS) do not usually require treatment, as the course of the disease is, in most cases, mild, and the prognosis is good. The purpose of this review is to underline the role of central autonomic network dysfunction in the development of Panayiotopoulos syndrome, as well as the possibility of using functional imaging techniques, especially functional magnetic resonance imaging (fMRI), in the diagnostic process. These methods could be crucial for understanding the pathogenesis of PS. More data arerequired to create algorithms that will be able to predict the exposure to various complications of PS. It also concerns the importance of electroencephalography (EEG) as a tool to distinguish Panayiotopoulos syndrome from other childhood epileptic syndromes and non-epileptic disorders.