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The Global Deterioration Scale for Primary Degenerative Dementia

The Global Deterioration Scale (GDS) provides caregivers an overview of the stages of cognitive function for those suffering from a primary degenerative dementia such as Alzheimer’s disease. It is broken down into 7 different stages. Stages 1-3 are the pre-dementia stages. Stages 4-7 are the dementia stages. Biginning in stage 5, an individual can no longer survive without assistance. Within the GDS, each stage is numbered (1-7), given a short title (i.e., Forgetfulness, Early Confusional, etc. followed by a brief listing of the characteristics for that stage. Caregivers can get a rough idea of where an individual is at in the disease process by observing that individual’s behavioral characteristics and comparing them to the GDS. For more specific assessments, use Brief Cognitive Rating Scale (BCRS) and the Functional Assessment Staging (FAST) measures.
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Clinical Criteria for Rasmussen´s Encephalitis

Rasmussen’s encephalitis (RE) is a rare chronic inflammatory brain disorder resulting in progressive neurodegeneration in one cerebral hemisphere. The inflammatory process is accompanied by progressive loss of function of the affected hemisphere, associated with drug-resistant partial epilepsy. The diagnosis is based on a range of clinical, electroencephalographic, radiological and biochemical arguments, without any specific formal marker, which makes the diagnosis of the disease complex, especially in its initial phase. Seizures are refractory to anti-seizures medication (ASM) and to classical immunomodulatory treatments.

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Common Types of Seizures in Adolescents and Adults

The clinical diagnosis of an epileptic seizure requires a detailed history taking and, ideally, an eyewitness account of the seizure. Evaluation with 12-lead electrocardiography is essential in a patient who has had a first seizure or an unexplained blackout spell.
Patients who have had an epileptic seizure should be informed about factors that may provoke seizures (e.g., sleep deprivation and alcohol use), the risk of a seizure occurring while driving or engaging in solitary activities, and the risks of harm from further seizures. Continue reading “Common Types of Seizures in Adolescents and Adults”

Clinical Aspects of the Diagnosis of Epileptic Seizures

The clinician must investigate and corroborate key features of the history that help to better characterize seizures while distinguishing epileptic seizures from nonepileptic events. The most common nonepileptic paroxysmal events during childhood and adolescence are syncope, psychogenic nonepileptic events (PNES), pallid and cyanotic breath holding spells, reflux/Sandifer syndrome, self-gratification disorders, and paroxysmal nonepileptic motor disorders of sleep among others. Continue reading “Clinical Aspects of the Diagnosis of Epileptic Seizures”

Awaji Criteria for Amyotrophic Lateral Sclerosis (ALS)

The Awaji recommendations for the use of electrodiagnostic studies in the diagnosis of amyotrophic lateral sclerosis (ALS) were proposed to enable earlier diagnosis of ALS to be achieved to meet an acknowledged need to obviate diagnostic delay. Continue reading “Awaji Criteria for Amyotrophic Lateral Sclerosis (ALS)”

Investigations to Evaluate Sensory Ganglionopathies

Among disorders that cause sensory loss, the most distinctive are those that affect the sensory ganglia. These disorders are called sensory neuronopathies or sensory ganglionopathies. The importance of sensory ganglia, in addition to the unique topography of sensory loss that occurs when they are diseased, lies in their close association with general medical disorders, and paraneoplastic disorders in particular. Furthermore, ganglia may be susceptible to autoimmune attack because of the fenestrated endothelial cells that form a permeable blood–nerve barrier. Continue reading “Investigations to Evaluate Sensory Ganglionopathies”

Revised Diagnostic Criteria for Neurofibromatosis Type 1 (NF1)

Neurofibromatosis type 1, inherited in an autosomal dominant pattern, is characterized by multiple café-au-lait macules (CALMs), skinfold freckling (more correctly termed lentiginous macules since they occur in non–sun exposed areas), iris Lisch nodules, tumors of the nervous system, and other features. Continue reading “Revised Diagnostic Criteria for Neurofibromatosis Type 1 (NF1)”

Diagnostic Criteria for Transient Ischemic Attack (TIA)

The diagnosis of transient ischemic attack (TIA) can be notoriously difficult, mainly because it is often solely based on history taking. Patients suspected of a TIA require an urgent assessment with timely start of antithrombotic therapy to reduce the risk of an early ischemic stroke. Continue reading “Diagnostic Criteria for Transient Ischemic Attack (TIA)”

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