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This is an inflammatory disorder that involves multiple small to medium-sized parenchymal and subarachnoid vessels and is characterized by chronic inflammation, multinucleated giant cells and destruction of the vessel wall. Affected individuals manifest a diffuse encephalopathic clinical picture, often with cognitive dysfunction; improvement occurs with steroid and immunosuppressive treatment.

Primary angiitis of the CNS (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 868


This describes reversible altered consciousness from head injury in the absence of contusion. The characteristic transient neurologic dysfunction includes loss of consciousness, temporary respiratory arrest, and loss of reflexes.

Concussion(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 869


Injury which may occur from collision of the brain with the skull opposite the site of impact.

Contrecoup injury(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 868


These are wedge shaped lesions of the brain parenchyma which occur secondary to trauma. The broad base of the wedge spans the surface and centered on the point of impact.

Contusions(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 868


Blood vessel involved in epidural hematoma.

Middle meningeal artery(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 870


Blood vessels involved in subdural hemorrhage.

Bridging veins(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 870


This is an extension of CNS tissue through a defect in the vertebral column, most commonly in the lumbosacral region. both The meninges and spinal cord parenchyma are included in the cystlike structure visible just above the buttocks.

Myelomeningocele(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 872


Herniation of the meninges through a defect in the vertebral column.

Meningocoele(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 872


This is a malformation of the anterior end of the neural tube, with absence of the brain and top of skull.

Anencephaly(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 872


This is a diverticulum of malformed CNS tissue extending through a defect in the cranium. It most often involves the occipital region or the posterior fossa.

Encephalocele(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 872


Characterized by an increased number of irregularly formed gyri that result in an irregular bumpy or cobblestone-like surface.

Polymicrogyria(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 872


Characterized by a disruption of the normal midline patterning. In severe forms the brain is not divided into hemispheres or lobes and may be associated with facial midline defects like cyclopia.

Holoprosencephaly (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 872


Consists of a small posterior fossa and a misshapen midline cerebellum with downward extension of vermis through the foramen magnum; hydrocephalus and a lumbar myelomeningocele are typically also present.

Arnold-Chiari malformation Type II(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 872


Consists of low-lying cerebellar tonsils which extend through the foramen magnum at the base of the skull. This can lead to obstruction of CSF flow and compression of the medulla, resulting in symptoms of headache or cranial nerve deficits.

Arnold-Chiari malformation type I(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 872


Cerebral malformation characterized by an enlarged posterior fossa. The cerebellar vermis is absent, and replaced by a large, midline cyst that is lined by ependyma and is contiguous with leptomeninges on its outer surface.

Dandy-Walker malformation (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 872


Spinal cord abnormality characterized by a discontinuous or confluent expansion of the ependyma-lined central canal of the cord.

Hydromyelia(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 873


CSF findings in bacterial meningitis.

Increased pressureabundant neurophilsprotein - elevatedglucose - reduced(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 874


This is an inflammatory process of the leptomeninges and CSF within the subarachnoid space.

Meningitis (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 874


In acute cases of this condition, an exudate is evident within the leptomeninges over the surface of the brain. On microscopic examination, neutrophils fill the entire subarachnoid space in severely affected areas or may be found predominantly around the leptomeningeal blood vessels.

Acute meningitis(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 874


A form of neurosyphilis, resulting from damage to the sensory nerves in the dorsal roots producing impaired joint position sense and resultant ataxia, loss of pain sensation, leading to skin and "Charcot joints". There is loss of both axons and myelin in the dorsal roots, with pallor and atrophy in the dorsal columns of the spinal cord.

Tabes dorsalis (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 875


Presents with generalized symptoms of headache, malaise, mental confusion, and vomiting. The subarachnoid space contains a gelatinous or fibrinous exudate, most often at the base of the brain, obliterating the cisterns and encasing cranial nerves. There may be discrete white granules scattered over the leptomeninges. Obliterative endarteritis may be seen.

Tuberculous meningitis (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 874


CSF findings in tuberculous meningitis.

Moderate pleiocytosis - predominantly monocytesprotein - elevatedglucose - moderately reduced/normal(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 874


Caused by invasion of the brain by Treponema pallidum and manifests as insidious but progressive loss of mental and physical functions with mood alterations (including delusions of grandeur), terminating in severe dementia.

Paretic neurosyphilis (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 874


This virus tends to localize in the paraventricular subependymal regions of the brain, which results in a severe hemorrhagic necrotizing ventriculoencephalitis and choroid plexitis.

Cytomegalovirus (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 877


These are discrete lesions with central liquefactive necrosis and a surrounding fibrous capsule. On microscopic examination, there is exuberant neovascularization around the necrosis that is responsible for the marked edema and formation of granulation tissue. Outside the fibrous capsule is a zone of reactive gliosis.

Brain Abscesses (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 876


The pathognomonic finding of this disease is a spongiform transformation of the cerebral cortex and deep gray matter structures (caudate, putamen); consists of a multifocal process that results in the uneven formation of small, empty, microscopic vacuoles of varying sizes within the neuropil and sometimes in the perikaryon of neurons.

Creutzfeldt-Jakob Disease (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 880


Viral encephalitis which involves inferior and medial regions of the temporal lobes and the orbital gyri of the frontal lobes. Perivascular inflammatory infiltrates are usually present, and Cowdry type A intranuclear viral inclusion bodies can be found in both neurons and glia.

Herpes Encephalitis(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 876


Reactivation of varicella in adults manifests as a painful, vesicular skin eruption in the distribution of one or a few dermatomes.

Shingles / Herpes Zoster(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 876


This lesion caused by JC virus, consist of patches of irregular, ill-defined destruction of the white matter that enlarge. Each lesion is an area of demyelination, in the center of which are scattered lipid-laden macrophages and a reduced number of axons.

Progressive Multifocal Leukoencephalopathy (PML) (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 877


These are predominantly benign tumors of adults, usually attached to the dura, and arising from the meningothelial cell of the arachnoid. Histologic patterns include syncitial, fibroblastic, psammomatous, secretory and microcystic.

Meningiomas(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 885