EPYLEPSY

Epilepsy (from the Ancient Greek epilpsa) is a common chronic neurological disorder characterized by recurrent unprovoked seizures.

History and stigma

The word epilepsy is derived from the Ancient Greek epilpsa, which was from epilambnein "to take hold of", which in turn was combined from ep "upon" and lambnein "to take". "Sacred Disease" 'disease of the assembly hall'

Classification

Epilepsies are classified in five ways:By their first cause (or etiology). By the observable manifestations of the seizures, known as semiology. By the location in the brain where the seizures originate. As a part of discrete, identifiable medical syndromes. By the event that triggers the seizures, as in primary reading epilepsy or musicogenic epilepsy.

The International League Against Epilepsy 1. ictal phenomenon,(pertaining to an epileptic seizure)2. seizure type,3. syndrome,4. etiology,5. impairment.

Precipitants

Certain epilepsy Photosensitive epilepsy Emotional stress, sleep deprivation, sleep itself, and febrile illness are examples of precipitants cited by patients with epilepsy. Catamenial epilepsy

Example of EEG

Epidemiology

The prevalence of active epilepsy is roughly in the range 510 per 1000 people. Up to 5% of people experience non febrile seizures at some point in life; epilepsy's lifetime prevalence is relatively high because most patients either stop having seizures or (less commonly) die of it. Epilepsy's approximate annual incidence rate is 4070 per 100,000 in industrialized countries and 100190 per 100,000 in resource-poor countries; socioeconomically deprived people are at higher risk.

Seizure types

Partial or focal seizures Generalized seizures Simple partial seizure Complex partial (psychomotor) seizure Generalized seizures: absence (petit mal), myoclonic, clonic, tonic, tonic-clonic (grand mal) and atonic seizures

Children may exhibit behaviors that are easily mistaken for epileptic seizures but are not caused by epilepsy Inattentive staring Benign shudders (among children younger than age 2, usually when they are tired or excited) Self-gratification behaviors (nodding, rocking, head banging) Conversion disorder (flailing and jerking of the head, often in response to severe personal stress such as physical abuse)

Epilepsy syndromes

Absence seizuresatonic seizuresbenign Rolandic epilepsy childhood absenceclonic seizures

complex partial seizuresfrontal lobe epilepsyFebrile seizuresInfantile spasmsJuvenile Myoclonic EpilepsyJuvenile Absence Epilepsylennox-gastaut syndromLandau-Kleffner Syndrome myoclonic seizuresProgressive Myoclonic EpilepsiesPsychogenic Seizures Reflex EpilepsySimple Partial seizures

Partial epilepsy

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--Syndromes are divided into localization-related epilepsies, generalized epilepsies, or epilepsies of unknown localization.-- Epilepsy syndromes are further divided by presumptive cause: idiopathic, symptomatic, and cryptogenic.

Below are some common seizure syndromes:Autosomal dominant nocturnal frontal lobe epilepsy Catamenial epilepsy Childhood absence epilepsy Frontal lobe epilepsy Juvenile myoclonic epilepsy Lennox-Gastaut syndrome Progressive myoclonic epilepsies Symptomatic localization-related epilepsies Temporal lobe epilepsyWest syndrome

Treatment

Epilepsy is usually treated with medication prescribed by a physician; primary caregivers, neurologists, and neurosurgeons all frequently care for people with epilepsy. In some cases the implantation of a stimulator of the vagus nerve, or a special diet can be helpful. Neurosurgical operations for epilepsy can be palliative, reducing the frequency or severity of seizures; or, in some patients, an operation can be curative.

status epilepticus a dangerous condition requiring hospitalization and emergency treatment.Objects should never be placed in a person's mouth by anybody - including paramedics - during a seizure as this could result in serious injury to either party. Despite common folklore, it is not possible for a person to swallow their own tongue during a seizure. However, it is possible that the person will bite their own tongue, especially if an object is placed in the mouth.

Pharmacologic treatment

anticonvulsant medication treatment will be lifelong carbamazepine (Tegretol), clorazepate (Tranxene), clonazepam (Klonopin), ethosuximide (Zarontin), felbamate (Felbatol), fosphenytoin (Cerebyx), gabapentin (Neurontin), lacosamide (Vimpat), lamotrigine (Lamictal), levetiracetam (Keppra), oxcarbazepine (Trileptal), phenobarbital (Luminal), phenytoin (Dilantin), pregabalin (Lyrica), primidone (Mysoline), tiagabine (Gabitril), topiramate (Topamax), valproate semisodium (Depakote), valproic acid (Depakene), and zonisamide (Zonegran)

Treatment Effectiveness - 50% of the patient treatment group had at least a 50% improvement in the rate of epileptic seizures. About 20% of patients with epilepsy continue to have breakthrough epileptic seizures despite best anticonvulsant treatment.88% of patients with epilepsy, in a European survey, reported at least one anticonvulsant related side effect: mood changes, sleepiness, or unsteadiness in gait, drug rashes, liver toxicity (hepatitis), or aplastic anemia, the consideration of teratogenicity.

Surgical treatment

Epilepsy surgery is an option for patients whose seizures remain resistant to treatment with anticonvulsant medications who also have symptomatic localization-related epilepsy

Resection of lesions like tumors or arteriovenous malformationsions Anterior temporal lobectomy Selective amygdal-ahippocampectomy callosotomy or commissurotomy Multiple subpial transection

Other treatment

Ketogenic dietElectrical stimulationVagus nerve stimulation Responsive Neurostimulator SystemDeep brain stimulation Noninvasive surgeryAvoidance therapyWarning systemsAlternative or complementary medicine

Pathophysiology

Mutations in several genes Epileptogenesis Abnormal mass of tissue in an area of the brain

Many jurisdictions forbid certain activities to persons suffering from epilepsy. In the U.S., people with epilepsy can drive if their seizures are controlled with treatment and they meet the licensing requirements in their state. In the UK, it is the responsibility of the patients to inform the Driver and Vehicle Licensing Agency if they have epilepsy. Persons with a history of epilepsy are usually prohibited from piloting aircraft, unless it can be shown beyond a reasonable doubt that future seizures are extremely improbable.

Vascular Dementia

Vascular dementia is the second most common form of dementia after Alzheimer disease (AD). The condition is not a single disease; it is a group of syndromes relating to different vascular mechanisms. Vascular dementia is preventable; therefore, early detection and an accurate diagnosis are important.

Vascular disease produces either focal or diffuse effects on the brain and causes cognitive decline. Focal cerebrovascular disease occurs secondary to thrombotic or embolic vascular occlusions. Common areas of the brain associated with cognitive decline are the white matter of the cerebral hemispheres and the deep gray nuclei, especially the striatum and the thalamus. Hypertension is the major cause of diffuse disease, and, in many patients, both focal and diffuse disease are observed together. The 3 most common mechanisms of vascular dementia are multiple cortical infarcts, a strategic single infarct, and small vessel disease.

multiinfarct dementia single-infarct dementia Binswanger diseaselacunar state vascular dementia

Frequency: Vascular dementia is the second most common cause of dementia in the United States and Europe, but it is the most common form in some parts of Asia. The prevalence rate of vascular dementia is 1.5% in Western countries and approximately 2.2% in Japan. In Japan, it accounts for 50% of all dementias that occur in individuals older than 65 years. In Europe, vascular dementia and mixed dementia account for approximately 20% and 40% of cases, respectively. In Latin America, 15% of all dementias are vascular. In community-based studies in Australia, the prevalence rate for vascular and mixed dementia is 13% and 28%, respectively. The prevalence rate of dementia is 9 times higher in patients who have had a stroke than in controls. One year after a stroke, 25% of patients develop new-onset dementia. Within 4 years following a stroke, the relative risk of incident dementia is 5.5%.

Sex: The prevalence of vascular dementia is higher in men than in women.

Age: Incidence increases with age.

Binswanger disease

The average age of onset is between the fourth and seventh decades of life, and 80% of patients have a history of hypertension.Patients also show progressive motor, cognitive, mood, and behavioral changes over a period of 5-10 years. Mood and behavioral changes are observed early and, in some patients, may be the presenting feature.Patients may be apathetic or abulic.Intellectual deficits are also observed early in the disease, and patients are frequently described as confused, inattentive, and vague.Patients with Binswanger dementia often have early-onset urinary incontinence and gait disturbances.

Cerebral autosomal dominant arteriopathy The onset of the disease occurs between the third and fourth decades of life.The clinical picture is similar to Binswanger disease but without a history of hypertension and risk factors for cerebrovascular disease.

Vascular dementia in general

Patients with vascular dementia commonly have mood and behavioral changes.Severe depression is more common in persons with vascular dementia than in those with AD.In some patients with lacunar state and Binswanger disease, such problems may be more prominent than intellectual deficits.Even psychotic symptoms, particularly delusions, have been described in patients with vascular dementia.

The Folstein Mini-Mental State Examination OrientationRegistrationAttentionRecallLanguage Scoring:

The DSM-IV diagnostic criteria The patient has developed multiple cognitive deficits manifesting as both (1) memory impairment and (2) one or more of the following cognitive disturbances: aphasia, apraxia, agnosia, and disturbance in executive functioning. The cognitive deficits in the above criteria cause significant impairment in social or occupational functioning and represent a significant decline from the previous level of functioning. Focal neurological signs and symptoms or radiological evidence indicative of cerebrovascular disease are present that are judged to be etiologically related to the dementia. The deficits do not occur exclusively during the course of delirium.

Causes: Risk factors for vascular dementia include hypertension, smoking, hypercholesterolemia, diabetes, and cardiovascular and cerebrovascular disease.

Medical Care Recent guidelines from the American Psychiatric Association provide both treatment principles and possible specific therapiesDrug treatment is primarily used to prevent further worsening of vascular dementia by treating the underlying disease such as hypertension and diabetes.The general management of dementia includes appropriate referral to community services, judgment and decision making regarding legal and ethical issues (eg, driving, competency, advance directives), and consideration of caregiver stress.

Diet In the Rotterdam study, an increased risk of vascular dementia was associated with total fat intake, whereas fish consumption was inversely related to dementia. Low levels of folate, vitamin B-6, and vitamin B-12 are associated with increased homocysteine levels, a risk factor for stroke.

Prognosis According to some studies, vascular dementia shortens life expectancy by approximately 50% in men, in persons with lower education, and in persons who perform worse on neuropsychological tests.The causes of death are due to complications of dementia, cardiovascular disease, and miscellaneous factors, including malignancy.

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