126 results found
Article
Cerebral atrophy
Cerebral atrophy is the morphological presentation of brain parenchymal volume loss that is frequently seen on cross-sectional imaging. Rather than being a primary diagnosis, it is the common endpoint for a range of disease processes that affect the central nervous system. Though often no identi...
Article
Sjögren-Larsson syndrome
Sjögren-Larsson syndrome is a rare inherited autosomal recessive neurocutaneous syndrome and leukodystrophy characterized by the clinical triad of ichthyosis, intellectual disability, and spastic diplegia or tetraplegia.
Epidemiology
Although considered very rare, the exact prevalence is not k...
Article
Alpers syndrome
Alpers syndrome, also known as Alpers-Huttenlocher syndrome or progressive cerebral poliodystrophy, is a rare childhood neurodegenerative POLG-related disorder. Along with Leigh syndrome, it is one of the commonest childhood mitochondrial disorders 1.
Epidemiology
Alpers syndrome is incredibl...
Article
Cortical vein sign
The cortical vein sign refers to the presence of superficial cortical veins seen on MRI and CT (particularly with contrast injection) traversing an enlarged subarachnoid space, differentiating it from the similar radiological appearance of a subdural hygroma.
Although initially proposed as a me...
Article
Benign enlargement of the subarachnoid space in infancy
Benign enlargement of the subarachnoid spaces in infancy (BESS or BESSI) also known as benign external hydrocephalus (BEH) is, as per the name, a benign enlargement of the subarachnoid spaces in infants. It usually involves the frontal lobe subarachnoid spaces, and it is characterized clinically...
Article
Mesial temporal sclerosis
Mesial temporal sclerosis, also commonly referred to as hippocampal sclerosis, is the most common association with intractable temporal lobe epilepsy 2,3,5. It is seen in up to 65% of autopsy studies, although significantly less in imaging.
Clinical presentation
Most patients present with temp...
Article
Sickle cell disease (cerebral manifestations)
Cerebral manifestations of sickle cell disease contribute significantly to the overall morbidity of the disease. Sickle cell disease is among the most common causes of stroke in the pediatric population.
For a general discussion of sickle cell disease, please refer to sickle cell disease.
Epid...
Article
Hypomelanosis of Ito
Hypomelanosis of Ito, also known as incontinentia pigmenti achromians, is the third most frequent phakomatosis, involving a wide spectrum of defects in multiple organ systems.
Epidemiology
The prevalence is unknown, being reported as between 1 per 8000 patients in a general pediatric hospital ...
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...
Article
Global cortical atrophy scale
The global cortical atrophy (GCA) scale, also known as the Pasquier scale, is a qualitative rating system developed to assess cerebral atrophy, especially in the context of neurodegenerative diseases. It evaluates atrophy in 13 brain regions assessed separately in each hemisphere and resulting i...
Article
Neurodegeneration with brain iron accumulation
Neurodegeneration with brain iron accumulation (NBIA), is a heterogeneous group of rare genetic disorders characterized by accumulation of iron in the basal ganglia, which results in variable neurological disorders including extrapyramidal symptoms, neuropsychiatric and visual symptoms.
Numerou...
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...
Article
Subcortical arteriosclerotic encephalopathy
Subcortical arteriosclerotic encephalopathy, also known as Binswanger disease or small vessel dementia, refers to slowly progressive, nonhereditary, exclusively white-matter vascular dementia.
Clinical presentation
Patients usually present with subcortical dementia symptoms including forgetful...
Article
CHEDDA syndrome
CHEDDA syndrome stands for congenital hypotonia, epilepsy, developmental delay and digital anomalies. Is a newly discovered neurodevelopmental syndrome associated with mutations in a conserved histidine-rich motif within Atrophin-1 (ATN-1).1
Epidemiology
CHEDDA syndrome is very rare, with only...
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
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
Cerebral fat embolism
Cerebral fat embolism is one manifestation of fat embolism syndrome, but can also rarely occur in isolation.
Epidemiology
Cerebral fat embolism typically occurs in patients with bony fractures (usually long bones of the lower limb) or following orthopedic or cardiac surgery 19. In particular, ...
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
Subdural hemorrhage
Subdural hemorrhage/hematoma (SDH) is a collection of blood accumulating in the subdural space. Subdural hemorrhage can happen in any age group, is mainly due to head trauma and CT scans are usually sufficient to make the diagnosis. Prognosis varies widely depending on the size and chronicity of...
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...
Unable to process the form. Check for errors and try again.