205 results found
Article
Hemorrhagic shock and encephalopathy syndrome
Hemorrhagic shock and encephalopathy syndrome (HSES) is a rare pediatric encephalopathy syndrome with a high mortality rate.
Epidemiology
Hemorrhagic shock and encephalopathy syndrome is considered rare, although the exact global incidence and prevalence is not known. The condition occurs in i...
Case
Artery of Percheron territory old infarction
Published
03 Feb 2024
95% complete
MRI
Article
Langerhans cell histiocytosis (CNS manifestations)
The central nervous system (CNS) is an uncommonly involved organ system in Langerhans cell histiocytosis (LCH). Involvement of the CNS is related but distinct from involvement of the skull or craniofacial structures, which are discussed separately in the article skeletal manifestations of LCH. F...
Article
Hot cross bun sign (pons)
The hot cross bun sign refers to the MRI appearance of the pons when T2 hyperintensity forms a cross on axial images, representing selective degeneration of transverse pontocerebellar tracts and median pontine raphe nuclei 1.
It has been described in a variety of neurodegenerative and other con...
Article
Autoimmune glial fibrillary acid protein (GFAP) astrocytopathy
Autoimmune glial fibrillary acid protein (GFAP) astrocytopathy, or simply GFAP astrocytopathy, is a rare inflammatory central nervous system (CNS) disorder.
Epidemiology
Given the rarity of the condition, epidemiological data pertaining to autoimmune GFAP astrocytopathy are not well establishe...
Article
Leigh syndrome
Leigh syndrome, also known as subacute necrotizing encephalomyelopathy (SNEM), is a mitochondrial disorder characterized by progressive neurodegeneration, mitochondrial dysfunction, and bilateral central nervous system lesions, that invariably leads to death, usually in childhood.
Epidemiology
...
Article
Moyamoya disease
Moyamoya disease is an idiopathic, non-inflammatory, non-atherosclerotic progressive vaso-occlusive disease involving the terminal supraclinoid internal carotid arteries and circle of Willis.
Terminology
The term moyamoya disease should be reserved for an idiopathic, sometimes familial, cond...
Case
Dyke-Davidoff-Masson syndrome
Published
25 Jan 2024
71% complete
CT
Article
Creutzfeldt-Jakob disease
Creutzfeldt-Jakob disease (CJD) is a transmissible spongiform encephalopathy that results in rapidly progressive dementia and death usually within a year from onset. The vast majority are sporadic, but familial and acquired forms are occasionally encountered.
On imaging, it classically manifest...
Article
Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia
Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP), also known as hereditary diffuse leukoencephalopathy with spheroids (HDLS) and pigmentary orthochromatic leukodystrophy (POLD), refers to a rare inherited autosomal dominant disease characterized by an adult-onset l...
Article
Tuberous sclerosis
Tuberous sclerosis (TS), also known as tuberous sclerosis complex (TSC) or Bourneville disease, is a phakomatosis (neurocutaneous disorder) characterized by the development of multiple benign tumors of the embryonic ectoderm (e.g. skin, eyes, and nervous system).
Epidemiology
Tuberous sclerosi...
Case
Disproportionately enlarged subarachnoid space hydrocephalus (DESH)
Published
05 Jan 2024
77% complete
CT
Article
Crossed cerebellar diaschisis
Crossed cerebellar diaschisis refers to a depression in function, metabolism, and perfusion affecting a cerebellar hemisphere occurring as a result of a contralateral focal supratentorial lesion, classically an infarct.
Clinical presentation
Other than neurological deficits and other clinica...
Article
HTLV-1-associated myelopathy
HTLV-1-associated myelopathy, also known as tropical spastic paraparesis, is primarily seen in Japan, Melanesia and the Caribbean and presents with chronic spastic paraparesis.
Terminology
This condition has been independently described in Japan (HTLV-1 associated myelopathy) and in the Caribb...
Article
Transependymal edema
Transependymal edema, also known as interstitial cerebral edema or periventricular lucency (PVL), is a type of cerebral edema that occurs with increased pressure within the cerebral ventricles. FLAIR is the most sensitive MRI sequence for detection.
Pathology
The ventricular ependymal lining i...
Article
Normal pressure hydrocephalus
Normal pressure hydrocephalus remains a controversial entity with often ambiguous imaging findings. It is classically characterized by the triad of gait apraxia/ataxia, urinary incontinence, and cognitive impairment, although not all patients with the condition have all three 31.
On imaging, it...
Article
Central nervous system curriculum
The central nervous system curriculum is one of our curriculum articles and aims to be a collection of articles that represent the core central nervous system knowledge.
Definition
Topics pertaining to the intracranial content (brain, pituitary, dura, intracranial vasculatures). There will be...
Case
Crossed cerebellar diaschisis in a patient with Dyke-Davidoff-Masson syndrome and a skull vault hemangioma
Published
01 Jan 2024
80% complete
MRI
Article
Autosomal dominant polycystic kidney disease
Autosomal dominant polycystic kidney disease (ADPKD), also sometimes referred to as "adult polycystic kidney disease", is an inherited form of adult cystic renal disease.
Epidemiology
Autosomal dominant polycystic kidney disease is one of the most common serious hereditary diseases, found in ...
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...
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