205 results found
Case
Cerebral fat embolism (type 2A) - confluent edema in white matter
Published
09 Mar 2022
68% complete
X-ray
CT
Article
Subacute combined degeneration of the cord
Subacute combined degeneration of the cord is caused by vitamin B12 deficiency.
Epidemiology
Most common in patients older than 40 years and especially older than 60 years 7.
Clinical presentation
The clinical presentation of subacute combined degeneration is usually with loss of vibration ...
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
Niemann-Pick disease type C
Niemann-Pick disease type c (NPD-C or just NPC) is an autosomal recessive lysosomal storage disorder classed under Niemann-Pick disease on account of clinical similarities, namely hepatosplenomegaly and variable involvement of the central nervous system.
Epidemiology
NPD-C is inherited as an ...
Article
Cerebrotendinous xanthomatosis
Cerebrotendinous xanthomatosis is an autosomal recessive lipid storage disorder caused by defects in sterol-27-hydroxylase enzyme in bile acid synthesis.
Clinical presentation
The clinical presentation is highly variable with many potential neurological and other/systemic clinical features 6.
...
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
Superficial siderosis of the central nervous system
Superficial siderosis of the central nervous system refers to a condition resulting from deposition of hemosiderin along the leptomeninges and subpial surfaces, particularly infratentorially, with eventual neurological dysfunction, presumably due to a source of slow and chronic/recurrent subarac...
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
Aceruloplasminemia
Aceruloplasminemia is an autosomal recessive type of neurodegeneration with brain iron accumulation and disorder of iron metabolism caused by a mutation in the ceruloplasmin (CP) gene resulting in the production of dysfunctional ceruloplasmin.
Epidemiology
Aceruloplasminemia is a very rare dis...
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...
Case
Elderly CT brain
Published
24 Nov 2014
56% complete
CT
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
Hemiconvulsion-hemiplegia epilepsy syndrome
Hemiconvulsion-hemiplegia epilepsy syndrome (HHE) is a clinical syndrome of infancy or early childhood that is associated with seizures, cerebral hemiatrophy and transient or permanent epilepsy.
It refers to the characteristic holohemispheric global atrophy of one hemisphere, that is independen...
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
Adult polyglucosan body disease
Adult polyglucosan body disease (APBD) is a very rare adult-onset form of glycogen storage disease type IV with characteristic clinicoradiological features.
Epidemiology
Adult polyglucosan body disease is considered very rare 1,2, but the exact incidence is not known and it may often be misdia...
Article
Progressive multifocal leukoencephalopathy
Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease which results from the reactivation of John Cunningham virus (JC virus) infecting oligodendrocytes in patients with compromised immune systems.
It is considered the most common clinical manifestation of JC virus infecti...
Article
Leukodystrophies
The leukodystrophies are a heterogeneous group of disorders that primarily affect the white matter of the central nervous system. They are particularly encountered in childhood as many are genetically determined and represent abnormalities in white matter metabolism. A number of leukodystrophies...
Article
Leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation
Leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation (LBSL) is a rare inherited autosomal recessive leukodystrophy characterized by slowly progressive pyramidal, cerebellar, and dorsal column dysfunction.
Epidemiology
Although considered rare, the exact prevalen...
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
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...
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