The epidemiology, etiology, evaluation, and diagnosis of early-onset dementia will be reviewed here. (medilib.ir)
Approximately 55 percent were felt to have a single progressive neurodegenerative etiology, predominantly Alzheimer disease (AD), frontotemporal dementia (FTD), dementia with Lewy bodies (DLB), dementia related to Parkinson disease (PD), and corticobasal degeneration (including corticobasal syndrome and many other phenotypes) [ 2 ]. (medilib.ir)
Epilepsy6
the prognosis depends on the underlying disorder, including its reversibility or treatability and the likelihood of developing epilepsy from it. (clinicalgate.com)
Epilepsy is a disorder of the brain characterized by an enduring predisposition to generate seizures and by the neurobiologic, cognitive, psychological, and social consequences of this condition. (clinicalgate.com)
Seizure disorder is a general term that is usually used to include any one of several disorders including epilepsy, febrile seizures, and possibly single seizures and seizures secondary to metabolic, infectious, or other etiologies (e.g., hypocalcemia, meningitis). (clinicalgate.com)
Idiopathic epilepsy is an epilepsy syndrome that is genetic or presumed genetic and in which there is no underlying disorder affecting development or other neurologic function (e.g., petit mal epilepsy). (clinicalgate.com)
Symptomatic epilepsy is an epilepsy syndrome caused by an underlying brain disorder (e.g., epilepsy secondary to tuberous sclerosis). (clinicalgate.com)
A cryptogenic epilepsy (also termed presumed symptomatic epilepsy ) is an epilepsy syndrome in which there is a presumed underlying brain disorder causing the epilepsy and affecting neurologic function, but the underlying disorder is not known. (clinicalgate.com)
Involuntary1
A seizure typically causes altered awareness, abnormal sensations, focal involuntary movements, or convulsions (widespread violent involuntary contraction of voluntary muscles). (msdmanuals.com)
Metabolic disorders1
Nonepileptic seizures are provoked by a temporary disorder or stressor (eg, metabolic disorders, central nervous system (CNS) infections, cardiovascular disorders, drug toxicity or withdrawal, psychogenic disorders). (msdmanuals.com)
Seizure3
Diagnosis may be clinical and involves results of neuroimaging, laboratory testing, and electroencephalography (EEG) for new-onset seizures or levels of antiseizure drugs (anticonvulsants) for previously diagnosed seizure disorders. (msdmanuals.com)
Neonatal Seizure Disorders Neonatal seizures are abnormal electrical discharges in the central nervous system of neonates and usually manifest as stereotyped muscular activity or autonomic changes. (msdmanuals.com)
An epileptic syndrome is a disorder that manifests one or more specific seizure types and has a specific age of onset and a specific prognosis. (clinicalgate.com)
Diagnosis2
Approach to the Patient With a Suspected Inherited Disorder of Metabolism Most inherited disorders of metabolism (inborn errors of metabolism) are rare, and therefore their diagnosis requires a high index of suspicion. (msdmanuals.com)
In particular, the differential diagnosis is much broader and often requires a more extensive evaluation that includes consideration of both common and rare disorders. (medilib.ir)
Focal1
presents with focal seizures with dysarthria, sialorrhea, dysphasia, and unilateral clonic or tonic-clonic movement of the mouth, or nocturnal focal to bilateral tonic-clonic seizures. (medlink.com)
Cognitive1
The remaining 45 percent of patients had the following etiologies: vascular-related cognitive change, alcohol-related cognitive change, Huntington disease (HD), cognitive impairment resulting from multiple sclerosis (MS), prion diseases, dementia related to Down syndrome (predominantly AD), and unknown/unclassified. (medilib.ir)
Specific1
Tic disorder not otherwise specified ( DSM-5 307.20): This category is for disorders characterized by tics that do not meet criteria for a specific tic disorder. (medscape.com)
Patients2
Psychogenic nonepileptic seizures (pseudoseizures) are symptoms that simulate seizures in patients with psychiatric disorders but that do not involve an abnormal electrical discharge in the brain. (msdmanuals.com)
An epidemiologic study in four London boroughs identified 185 patients with an onset of dementia between 30 to 64 years of age [ 2 ]. (medilib.ir)
Brain1
Heterozygous de novo pathogenic variants in MAPK8IP3 result in a neurodevelopmental disorder with or without brain abnormalities and possible axonal peripheral neuropathy. (bvsalud.org)
Years3
Examples include tics lasting less than 4 weeks or tics with an onset after age 18 years. (medscape.com)
Early-onset dementia - Although terminology varies, we consider early-onset dementia to refer to cases of dementia occurring in adults ranging from 18 to 65 years of age. (medilib.ir)
Some authors further subdivide early-onset dementia, using young-onset dementia to refer to cases with an age of onset between 18 and 45 years. (medilib.ir)
Seizures8
Diagnosis may be clinical and involves results of neuroimaging, laboratory testing, and electroencephalography (EEG) for new-onset seizures or levels of antiseizure drugs (anticonvulsants) for previously diagnosed seizure disorders. (msdmanuals.com)
Neonatal Seizure Disorders Neonatal seizures are abnormal electrical discharges in the central nervous system of neonates and usually manifest as stereotyped muscular activity or autonomic changes. (msdmanuals.com)
Nonepileptic seizures are provoked by a temporary disorder or stressor (eg, metabolic disorders, central nervous system (CNS) infections, cardiovascular disorders, drug toxicity or withdrawal, psychogenic disorders). (msdmanuals.com)
Psychogenic nonepileptic seizures (pseudoseizures) are symptoms that simulate seizures in patients with psychiatric disorders but that do not involve an abnormal electrical discharge in the brain. (msdmanuals.com)
The increasing interest in the last century and technologic development provided complementary information about the disorder and started to be characterized not only by its basic epiphenomenon, the seizures, its considered "a brain disorder" characterized by a predisposition to generate epileptic seizures with the neurobiological, cognitive, psychological, and social consequences of it. (surgicalneurologyint.com)
It can be conceptualized as a temporary and reversible change in brain functioning that has not been caused by fever, drugs, or metabolic disorders and is expressed by repeated seizures at irregular time intervals. (surgicalneurologyint.com)
SCN1A seizure disorders encompass a spectrum that ranges from simple febrile seizures and generalized epilepsy with febrile seizures plus (GEFS+) at the mild end to Dravet syndrome and intractable childhood epilepsy with generalized tonic-clonic seizures (ICE-GTC) at the severe end. (nih.gov)
Anti-LGI1 encephalitis is preceded by faciobrachial dystonic seizures (FBDS), which are specific involuntary movements and frequently complicated by hyponatremia due to the syndrome of inappropriate secretion of antidiuretic hormone (SIADH). (bvsalud.org)
Neurologic1
C26733 Cranial Nerve Disorder C103171 Recurrent Laryngeal Nerve Injury C118464 Pediatric Adverse Events Terminology C35078 Tourette Syndrome Tourette Syndrome Gilles de la Tourette syndrome A neurologic disorder caused by defective metabolism of the neurotransmitters in the brain. (nih.gov)
Hyperkinetic2
They vary from simple arousals from sleep to dramatic, often hyperkinetic events with tonic or dystonic features. (nih.gov)
Developmental and epileptic encephalopathy-6B (DEE6B) is a severe neurodevelopmental disorder characterized by early-infantile seizure onset, profoundly impaired intellectual development, and a hyperkinetic movement disorder. (nih.gov)
Lesions1
Common pitfalls in AE misdiagnosis are mesiotemporal lesions (predominantly with atypical features) and false-positive serum antibodies. (bvsalud.org)
Seizure disorders1
The phenotype of SCN1A seizure disorders can vary even within the same family. (nih.gov)
Tics3
Tic disorder not otherwise specified ( DSM-5 307.20): This category is for disorders characterized by tics that do not meet criteria for a specific tic disorder. (medscape.com)
Examples include tics lasting less than 4 weeks or tics with an onset after age 18 years. (medscape.com)
A neurological disorder presenting in childhood that is characterized by both motor and phonic tics that occur daily or nearly daily for at least a year and are not attributed to an identifiable cause. (nih.gov)
Encephalitis1
Anti-Caspr2 encephalitis (Morvan's syndrome) is associated with limbic symptoms, severe autonomic disorders, muscle cramps and burning extremity pain due to peripheral nerve hyperexcitability. (bvsalud.org)
Neurodegenerative1
Much smaller amounts of iron accumulation have also been associated with neurodegenerative disorders such as Alzheimer disease, Parkinson disease, and multiple sclerosis. (medlink.com)
Cerebellar1
Exclusion criteria were pure cerebellar or peripheral nerve system disorders. (bvsalud.org)
Encompass1
Neurodegeneration with brain iron accumulation may encompass a larger number of disorders. (medlink.com)
Clinical1
METHODS: In this retrospective cohort study, patients referred to our national referral center with suspicion of AE and specific neuroinflammatory disorders with similar clinical presentations were included from July 2016 to December 2019. (bvsalud.org)
Ranges1
Age of onset ranges from infancy to adulthood. (nih.gov)
Metabolism1
Approach to the Patient With a Suspected Inherited Disorder of Metabolism Most inherited disorders of metabolism (inborn errors of metabolism) are rare, and therefore their diagnosis requires a high index of suspicion. (msdmanuals.com)
Voluntary1
An action tremor that occurs with any form of voluntary movement including visually or non-visually guided actions, such as speaking, pouring water into a cup, or finger-to-nose testing. (medscape.com)
Features1
The classic phenotypic features of pantothenate kinase-associated neurodegeneration include early onset and rapidly progressive disease. (medlink.com)
Vocal1
C26726 Infectious Disease C118420 Otolaryngologic Disorder C118464 Pediatric Adverse Events Terminology C47814 Vocal Cord Paralysis Vocal Cord Paralysis Paralysis of the muscles of one or both vocal cords. (nih.gov)
Vary1
Within a family, the manifestations of the disorder may vary considerably. (nih.gov)
Muscle2
This causes a variety of elementary neurological deficits including asynergy (lack of coordination between muscles, limbs and joints), dysmetria (lack of ability to judge distances that can lead to under- or overshoot in grasping movements), and dysdiadochokinesia (inability to perform rapid movements requiring antagonizing muscle groups to be switched on and off repeatedly). (nih.gov)
However, movements may be strung together and thus may appear to flow randomly from one muscle group to another. (nih.gov)
Simple1
Includes dynamic or intention tremor, which occurs with target-directed movements, and simple kinetic tremor, which is present with non-targeted directed actions. (medscape.com)