Inherited Metabolic Disorders Main Text Preamble Inherited metabolic disorders—a.k.a. inborn errors of metabolism—represent conditions in which a genetic defect leads to a deficiency of a protein (e.g., enzyme or nonenzyme…
Demyelinating and Inflammatory Diseases Main Text Preamble Once considered an “immune-privileged” site sequestered beyond the blood-brain barrier, we now know there is active and continuous immunologic surveillance in the CNS….
Toxic Encephalopathy Main Text Preamble The list of toxins and poisons that affect the CNS is long and continues to grow. Some agents are deliberately injected, inhaled, or ingested, whereas…
Dementias and Brain Degenerations Main Text Preamble One in three adults over 85 years old suffers from Alzheimer disease or other forms of dementia. New treatments to slow progression of…
Mesenchymal, Nonmeningothelial Tumors Main Text Preamble The terminology and pathologic features of benign and malignant mesenchymal, nonmeningothelial neoplasms originating in the CNS correspond to soft tissue or bone tumors found…
Ependymal Tumors Main Text Preamble Overall, ependymomas represent 2-3% of all primary CNS neoplasms. Approximately 30% affect children and adolescents, although ependymal neoplasms occur at all ages, encompass multiple tumor…
Secondary Effects and Sequelae of CNS Trauma Main Text Preamble Traumatic brain injury is not a single “one and done” event. A veritable “cascade” of adverse pathophysiologic events continues to…
Embryonal Tumors Main Text Preamble The rapidly evolving molecular classification of brain tumors has fundamentally changed the understanding of embryonal neoplasms. In the 2021 WHO schema, the classification of the…
Commissural and Cortical Maldevelopment Main Text Preamble Corpus callosum dysgenesis and malformations of cortical development are two of the most important congenital brain anomalies. Anomalies of the cerebral commissures are…
Vascular Neurocutaneous Syndromes Main Text Preamble A number of syndromes with prominent cutaneous manifestations occur without associated neoplasms and can be inherited or occur spontaneously. Many of these are disorders…
