126 results found
Article
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an autosomal dominant microvasculopathy characterized by recurrent lacunar and subcortical white matter ischemic strokes and vascular dementia in young and middle age patients without known va...
Article
Systemic lupus erythematosus (CNS manifestations)
Central nervous system manifestations of systemic lupus erythematosus (CNS lupus), also known as neuropsychiatric systemic lupus erythematosus (NPSLE), describe a very diverse range of neuropsychiatric manifestations that are secondary to systemic lupus erythematosus (SLE) in the central nervous...
Article
HIV-associated dementia
HIV-associated dementia (HAD), previously referred to as AIDS dementia complex (ADC), corresponds to a neurological clinical syndrome seen in patients with HIV infection. The associated imaging appearance is generally referred to as HIV encephalopathy.
Terminology
The terms HIV dementia comple...
Article
Uremic encephalopathy
Uremic encephalopathy is an acquired toxic syndrome characterized by delirium in patients with untreated or inadequately treated acute or chronic kidney disease 13. Uremic encephalopathy is often associated with lethargy and confusion in the acute phase, which can progress to seizures, coma, or ...
Article
Nasu-Hakola disease
Nasu-Hakola disease, also known as polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy, is a rare inherited neuropsychiatric disorder which in addition to cognitive impairment also demonstrates bone cysts.
Epidemiology
Although the exact incidence is not known, the c...
Article
Visual agnosia
Visual agnosia is a special type of agnosia where patients are unable to recognize objects despite having knowledge about them and being able to visually perceive them.
It typically occurs when there is damage to the ventral occipitotemporal pathway, such as in posterior cerebral atrophy (Benso...
Article
Hyperostosis frontalis interna
Hyperostosis frontalis interna is characterized by benign overgrowth of the inner table of the frontal bone. The etiology is unknown. The condition is generally an incidental finding of no clinical significance. It is typically bilateral and symmetrical and may extend to involve the parietal bon...
Article
Cella media index
The cella media index (also known as Schiersmann's index) is one of the methods used to assess ventricle size with respect to brain tissue and cerebral atrophy. It is the ratio of biparietal diameter of skull to maximum external diameter of bodies of the lateral ventricles (cella media) 1.
A no...
Article
Megalencephalic leukoencephalopathy with subcortical cysts
Megalencephalic leukoencephalopathy with subcortical cysts, also known as Van der Knaap disease, refers to a rare inherited autosomal recessive disease characterized by diffuse subcortical leukoencephalopathy associated with white matter cystic degeneration.
Epidemiology
The age at symptoms m...
Article
Hurler syndrome
Hurler syndrome is one of the mucopolysaccharidoses (MPS type I).
Epidemiology
The estimated incidence is ~1:100,000.
Clinical presentation
It manifests in the first years of life with intellectual disability, corneal clouding, deafness, and cardiac disease. Death usually occurs within the f...
Article
Anorexia nervosa
Anorexia nervosa is a psychiatric disorder characterized by distorted self-perception of body weight leading to starvation, obsession with remaining underweight, and an excessive fear of gaining weight. One in five patients with anorexia dies due to complications of the disease.
Epidemiology
T...
Article
Anorexia nervosa (CNS manifestations)
The CNS manifestations of anorexia nervosa are common but varied with most of the imaging features non-specific in their own right.
For a general discussion, and for links to other system specific manifestations, please refer to the article on anorexia nervosa.
Epidemiology
Studies have iden...
Article
Hydrocephalus
Hydrocephalus denotes an increase in the volume of CSF, causing the distension of cerebral ventricles (ventriculomegaly) due to insufficient passage of the CSF from its point of production in the ventricles into the systemic circulation 7.
Terminology
Although hydrocephalus is typically referr...
Article
Dementia
Dementia, relabelled major neurocognitive disorder in the Diagnostic and Statistical Manual of Mental Disorders (DSM-5), is a clinical syndrome defined by progressive decline in one or more cognitive domains relative to a person's premorbid functional level. To fulfill the criteria for dementia,...
Article
Syphilis
Syphilis is the result of infection with the gram-negative spirochete Treponema pallidum, subspecies pallidum. It results in a heterogeneous spectrum of disease with many systems that can potentially be involved, which are discussed separately.
Epidemiology
Despite the discovery of penicillin...
Article
Kabuki syndrome
Kabuki syndrome (Niikawa-Kuroki syndrome) is a rare polydysplasia that is thought to be more common in Japan.
Epidemiology
The estimated incidence is 1:32.000 in Japan. It is sporadically seen elsewhere in the world. The reason for the disparity in incidence is controversial.
Clinical present...
Article
Myoclonic epilepsy with red ragged fibers (MERRF)
Myoclonic epilepsy with red ragged fibers (MERRF) is a rare multisystem mitochondrial disorder.
Clinical presentation
Patients usually present in late adolescence or early adulthood with 1-3
myoclonus
epilepsy
cerebellar ataxia
intellectual disability
vision and/or hearing loss
cardiomyo...
Article
Kwashiorkor
Kwashiorkor is a condition characterized by bilateral edema caused by protein deficiency.
Epidemiology
The largest population at risk for the condition are children who have begun weaning from breast milk or formula towards diets without sufficient protein. Therefore, kwashiorkor often affects...
Article
Incontinentia pigmenti
Incontinentia pigmenti, also known as Bloch-Sulzberger syndrome, is a rare condition that can affect many body systems, especially the skin. As an X-linked dominant genetic disorder, it occurs much more often in females than in males.
Epidemiology
Incontinentia pigmenti is rare and the true pr...
Article
Neuroferritinopathy
Neuroferritinopathy, also known as neurodegeneration with brain iron accumulation type 2 (NBIA type 2), is a disorder of iron metabolism caused by a mutation in the ferritin light chain 1 gene (FTL1) on chromosome 19.
Epidemiology
Neuroferritinopathy is a rare disorder first described in 2001 ...
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