Seizure syndrome

Infantile spasms A seizure syndrome that occurs in infants less than 1 year of age. It is characterized by a specific EEG pattern and spasms or jitters and is also known as West s syndrome. Infants with infantile spasms often develop other seizure types and epilepsies later in life. 

Neurological symptoms were observed in 12-25% of liver-transplant patients and in 29% of bone marrow transplant patients, but severe neurotoxicity occurred only in about 1% (18,19/21). They usually appeared within the first month of treatment, but were sometimes delayed (19). Particular attention should be paid to prompt recognition of severe neurotoxicity, because abnormalities of the white matter can occur. Patients usually improved rapidly after temporary ciclosporin withdrawal or dosage reduction, and tacrolimus has sometimes been used successfully instead (SEDA-21, 383) (18). However, recurrence of seizures and persistent electroencephalographic abnormahties were found in 46 and 70% of pediatric transplant patients respectively who had had ciclosporin acute encephalopathy and seizure syndrome and who were followed-up for 49 months (22). 

Gleeson JG, duPlessis AJ, Barnes PD, Riviello JJ Jr. Cyclosporin A acute encephalopathy and seizure syndrome in childhood clinical features and risk of seizure recurrence. J Child Neurol 1998 13(7) 336 4. 

Accurate diagnosis and classification of seizure/syndrome type is critical to selection of appropriate pharmacotherapy. 

Myoclonic syndromes Myoclonic seizure syndromes are usually treated with valproic acid. Clonazepam can be effective, but the high doses required cause drowsiness. Lamotrigine is also reported to be effective in myoclonic syndromes in children. Felbamate has been used adjunctively with the primary drugs but has hematotoxic and hepatotoxic potential. 

Epilepsy is a heterogeneous group of syndromes characterized by abnormal, rhythmic electrical activity of the brain or parts of the brain. The term epilepsy is reserved for chronic diseases, while a single, isolated seizure does not justify the diagnosis of epilepsy. 

These drug are used cautiously in patients with renal or hepatic disease, bladder obstruction, seizure disorders, sick sinus syndrome, gastrointestinal bleeding, and asthma Individuals with a history of ulcer disease may have a recurrence of the bleeding. 

This complex consists of four subunits, all of which are encoded on nuclear DNA, synthesized on cytosolic ribosomes, and transported into mitochondria. The succinate dehydrogenase (SDH) component of the complex oxidizes succinate to fumarate with transfer of electrons via its prosthetic group, FAD, to ubiquinone. It is unique in that it participates both in the respiratory chain and in the tricarboxylic acid (TC A) cycle. Defects of complex II are rare and only about 10 cases have been reported to date. Clinical syndromes include myopathy, but the major presenting features are often encephalopathy, with seizures and psychomotor retardation. Succinate oxidation is severely impaired (Figure 11). 

Other sedative-hypnotic medications, such as barbiturates, may play a useful role in severe withdrawal from this group of drugs. For example, in a case series of GBL withdrawal, use of intravenous pentobarbital in the range of 1-2 mg/kg/hour lowered the total requirement for intravenous lorazepam (Sivilotti et al. 2001). Antipsychotic medications are often used to reduce psychotic agitation. However, because antipsychotic medications lower the seizure threshold and may contribute to loss of central control of temperature leading to hyperthermia or neuroleptic malignant syndrome (NMS), they are not indicated as first-line medications for GHB withdrawal delirium (Dyer and Roth 2001 McDaniel and Miotto 2001 Sharma et al. 2001). If anti-... 

In overdose, ketamine may lead to hyperthermia, seizures, hypertensive crisis, coma, and even death. These symptoms are generally thought to be caused by ketamine s catecholaminergic effects (Reich and Silvay 1989). Ketamine is physically addicting, with a described withdrawal syndrome. 

Specific syndromes (epileptic seizures that may complicate many diseases, e.g., Ramsay-Hunt syndrome, Unverricht s disease)... 

Classification of epilepsies and epilepsy syndromes is helpful in determining appropriate pharmacotherapy. This classification scheme is based on the type of seizures a patient has and an attempt to identify the etiology of the epilepsy or epilepsy syndrome. 

Idiopathic epilepsies These syndromes are thought to be due to genetic alterations, but the underlying etiology is not identified. Neurologic functions are completely normal apart from the occurrence of seizures. 

A complete description of a patient s epilepsy should include the seizure type with the epilepsy or syndrome type (i.e., idiopathic, symptomatic, or cryptogenic). 

Juvenile myoclonic epilepsy (JME) A primary generalized epilepsy syndrome that usually starts in the early to middle teenage years and has a strong familial component. Patients have myoclonic jerks and tonic-clonic seizures and may also have absence seizures. 

Lennox-Gostaut syndrome (LGS) Patients with this syndrome have cognitive dysfunction and mental retardation. Their seizures usually consist of a combination of tonic-clonic, absence, atonic, and myoclonic seizures. 

Once it is concluded that the patient has seizures, the type of seizure and epilepsy syndrome, if any, must be determined. Proper identification and classification of the seizure type is most helpful in selecting appropriate pharmacotherapy. Without an accurate classification of the seizure type, it is possible to select a medication that is ineffective or even harmful to the patient. 

This is the most commonly observed syndrome, and as the name denotes, is not complicated by seizures, delirium tremens (DTs),... 

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