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 some overlap between this curriculum and the head and neck curriculum and the pediatric curriculum. 

Anatomy and development

An understanding of the anatomy of relevant structures is essential. Core anatomical topics include:

• anatomy of the brain

  • cerebrum

  • basal ganglia

  • brainstem

  • cerebellum

  • pituitary gland

  • Rathke pouch

• vascular anatomy of the brain

  • cerebral vascular territories

  • circle of Willis

  • intracranial arteries

    • anterior cerebral artery (ACA)

    • middle cerebral artery (MCA)

    • posterior cerebral artery (PCA)

    • superior cerebellar artery (SCA)

    • anterior inferior cerebellar artery (AICA)

    • posterior inferior cerebellar artery (PICA)

    • recurrent artery of Heubner

  • intracranial arteries-variants

    • azygous ACA

  • cerebral veins

    • dural venous sinuses

      • superior sagittal sinus

      • inferior sagittal sinus

      • straight sinus

      • transverse sinus

      • sigmoid sinus

      • confluence of sinuses

    • superficial cerebral veins

      • superficial middle cerebral vein (Sylvian vein)

      • superior anastomotic vein (of Trolard)

      • inferior anastomotic vein (of Labbe)

    • deep cerebral veins

• development of the central nervous system

  • normal myelination

Radiological examinations

Imaging techniques relevant to imaging of the central nervous system include:

• MRI

• CT and CT angiography (CTA)

• digital subtraction angiography (DSA)

• cranial ultrasound

• skull x-ray

Neonatal hypoxic-ischemic brain injuries

• hydranencephaly and porencephaly

• periventricular-intraventricular hemorrhage (PIVH)

• periventricular leukomalacia (PVL) and subcortical leukomalacia

• hypoxic-ischemic encephalopathy of the term newborn

  • multicystic encephalomalacia

  • ulegyria 

Malformations and genetic syndromes

Cortical malformations

Proliferative disorders

• microencephaly 

• megalencephaly

• macrocephaly

• cortical tubers

• cortical dysplasias

  • balloon-cell dysplasia

• gangliogliomas 

• gangliocytomas

• dysembryoplastic neuroepithelial tumors (DNET)

Migration disorders

• lissencephaly

• heterotopia

• cobblestone complex

Organization disorders

• polymicrogyria

• schizencephaly

• focal cortical dysplasia

  • Taylor type cortical dysplasia

    • cortical dysplasia(s) without balloon-cells

• microdysgenesis

For more detail see malformations of cortical development classification. 

Cerebellar malformations

• Dandy-Walker complex 

• cerebellar hypoplasia 

• cerebellar agenesis

• rhombencephalosynapsis 

• Lhermitte-Duclos syndrome

• Joubert syndrome

Dysraphic malformations

• myelomeningoceles

• encephaloceles

Other malformations

• hypo or agenesis of the corpus callosum

• holoprosencephaly

• septo-optic dysplasia

• arhinencephaly

• Chiari malformations 

  • Chiari I

  • Chiari II

  • Chiari III

Phakomatoses

• neurofibromatosis

  • neurofibromatosis type 1 (NF1) (von Recklinghausen disease)

  • neurofibromatosis type 2 (NF2)

• tuberous sclerosis (Bourneville-Pringle disease)

  • tuberous sclerosis diagnostic criteria

• ataxia telangiectasia

• Sturge-Weber syndrome (encephalotrigeminal angiomatosis)

• von Hippel-Lindau disease (retinocerebellar angiomatosis)

• incontinentia pigmenti (Bloch-Sulzberger syndrome)

• basal cell nevus syndrome (Gorlin-Goltz syndrome)

• Wyburn-Mason syndrome (Bonnet-Dechaume-Blanc syndrome)

• encephalocraniocutaneous lipomatosis 

• hypomelanosis of Ito

• Nijmegen breakage syndrome

• epidermal nevus syndrome

  • Schimmelpenning syndrome

  • phacomatosis pigmentokeratotica

  • nevus comedonicus syndrome

  • angora hair nevus syndrome

  • Becker nevus syndrome

  • Proteus syndrome

  • type 2 segmental Cowden disease

  • CHILD syndrome

  • FGFR3 epidermal nevus syndrome

• ​neurocutaneous melanosis 

• progressive facial hemiatrophy (Parry-Romberg syndrome)

• PHACE syndrome

• Cowden disease/COLD syndrome

• Gomez-Lopez-Hernandez syndrome

Metabolic and toxic CNS diseases

Metabolic encephalopathies

Inborn errors of metabolism

• Wilson disease

• leukodystrophies

  • lysosomal storage diseases

    • globoid cell leukodystrophy (Krabbe disease)

    • Fabry disease

    • metachromatic leukodystrophy

    • mucopolysaccharidoses

    • Niemann-Pick disease

  • peroxisomal disorders

    • adrenoleukodystrophies

    • Zellweger syndrome

  • mitochondrial dysfunction

    • Kearns-Sayre syndrome

    • Leigh disease

    • MELAS

    • MERRF

  • amino acid metabolism disorders

    • Canavan disease

  • unknown mechanism / others

    • adult-onset leukoencephalopathy with axonal spheroids and pigmented glia

    • Alexander disease

    • congenital muscular dystrophies (Fukuyama type)

    • cystic leukoencephalopathy without megalencephaly

    • glutaric aciduria

      • type I glutaric aciduria

      • type II glutaric aciduria 

    • L-2-hydroxyglutaric aciduria

    • Labrune syndrome

    • leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation

    • megalencephalic leukoencephalopathy with subcortical cysts

    • Nasu Hakola disease

    • Pelizaeus-Merzbacher disease

Toxic encephalopathy

Alcohol use-related

• Marchiafava-Bignami disease

• Wernicke encephalopathy (vitamin B₁ deficiency)

• osmotic myelinolysis (central pontine myelinolysis)

• hepatic encephalopathy

Others

• carbon monoxide intoxication

• toluene intoxication

• methanol intoxication

• ethylene glycol intoxication

• inhaled heroin pyrolysate ("chasing the dragon")

• methotrexate-related leukoencephalopathy 

Inflammatory demyelinating diseases

• clinically isolated syndrome (CIS)

• radiologically isolated syndrome (RIS)

• multiple sclerosis (MS)

  • variants

    • tumefactive multiple sclerosis

    • acute malignant Marburg type

    • Schilder type (diffuse cerebral sclerosis)

    • Balo concentric sclerosis (BCS)

    • opticospinal multiple sclerosis (OSMS)

• neuromyelitis optica (NMO) (Devic disease)

• acute disseminated encephalomyelitis (ADEM) and acute hemorrhagic encephalomyelitis (AHEM)

• tumefactive demyelinating lesions

• transverse myelitis

• chronic inflammatory demyelinating polyneuropathy (CIDP)

• Guillain-Barre Syndrome (GBS)

  • acute inflammatory demyelinating polyradiculoneuropathy (AIDP)

  • axonal subtypes

    • acute motor axonal neuropathy (AMAN) (Chinese paralytic syndrome)

    • acute motor-sensory axonal neuropathy (AMSAN)

  • regional GBS syndromes

    • Miller Fisher variant

Epilepsy

• general value of CT, MRI and fMRI

• mesial temporal sclerosis (MTS)

Brain tumors and tumor-like lesions

• intra vs extra-axial location

• DWI, PWI, MRS of brain tumors

Classification

• WHO classification of CNS neoplasms

1: Neuroepithelial tumors

• astrocytoma (WHO grade II)

• anaplastic (malignant) astrocytoma (WHO grade III)

• glioblastoma (WHO grade IV)

• pilocytic astrocytoma

• subependymal giant cell astrocytoma

• pleomorphic xanthoastrocytoma

• oligodendroglioma

• ependymoma

• subependymoma

• mixed gliomas

• gliomatosis cerebri (WHO grade IV)

• choroid plexus papilloma

• choroid plexus carcinoma

Neuronal and mixed neuronal-glial tumors

• gangliocytoma

• ganglioglioma

• central neurocytoma

• dysembryoplastic neuroepithelial tumor (DNET)

• olfactory neuroblastoma (esthesioneuroblastoma)

Pineal tumors

• pineocytoma

• pineoblastoma

Embryonal tumors

• medulloblastoma

• cerebral primitive neuroectodermal tumor (PNET)

2: Tumors of the peripheral nerves

• schwannoma (neurinoma, neurilemoma) 

• schwannomatosis

• neurofibroma

  • circumscribed neurofibroma

  • plexiform neurofibroma

• malignant schwannoma

3: Meningeal tumors

• meningioma

• atypical meningioma

• anaplastic (malignant) meningioma

• non-meningothelial primaries

  • CNS hemangiopericytoma

  • CNS fibrous histiocytoma

  • meningeal sarcomatosis

  • CNS malignant melanoma

4: Lymphatic and hematopoietic tumors

• primary malignant lymphoma

• plasmacytoma (CNS involvement)

5: Germ cell tumors

• germinoma 

• CNS embryonal carcinoma

• CNS yolk sac tumor

• choriocarcinoma 

• intracranial teratoma

• mixed germ cell tumors

6: Tumors of the sellar region

• pituitary adenoma 

• pituitary carcinoma

• craniopharyngioma

7: Metastases to the brain

• cerebral metastases

8: Intracranial extensions from the skull

• paraganglioma (chemodectoma)

• jugular paraganglioma

• chordoma

• chondroma 

• chondrosarcoma

• carcinoma

• metastases

9: Non-neoplastic mass lesions and cysts

• epidermoid 

• dermoid

• colloid cyst of the third ventricle

• neuroglial cyst

• Rathke cleft cyst

• granular cell tumor (choristoma, pituicytoma) 

• hypothalamic neuronal hamartoma

• nasal glial heterotopia 

• plasma cell granuloma

Vascular Lesions

Stroke: diagnosis and therapy

• pathophysiology of ischemic and hemorrhagic stroke

• cardiovascular conditions leading to ischemia and stroke

• imaging patterns of stroke

• early CT signs of acute cerebral ischemia

• conventional MR Imaging findings

• CT perfusion, CTA

• diffusion-weighted MR imaging (DWI)

• MRA and MR perfusion

• ischemic penumbra

• stroke therapy and outcome

Spontaneous intracranial hemorrhage

• aneurysm rupture

• other non-traumatic causes of subarachnoid hemorrhage

• subdural hematoma

• epidural hematoma

• intracerebral hematoma

Vascular anomalies and malformations

• hemangioma

• arteriovenous malformation

• dural AV fistula

• carotid sinus cavernous fistula

• aneurysms 

  • arterial

  • venous

• developmental venous anomaly (DVA)

Venous thrombosis

• dural venous sinus thrombosis

• deep vein thrombosis

Vascular neurointerventional procedures

• indications

• basic techniques

Infectious diseases of the CNS

Classification 

• meninges 

  • pachymeningitis

  • epidural abscess

  • leptomeningitis (meningitis) 

  • subdural empyema

• cerebritis and brain abscess

  • septic-embolic focal encephalitis

  • chronic encephalitides

• ventriculitis (ependymitis)

• choroid plexitis

• rhombencephalitis

• cerebellitis

Granulomatous inflammatory diseases

• CNS tuberculosis

• neurosarcoidosis

Bacterial infections

• CNS listeriosis

  • listeria rhombencephalitis

• CNS nocardiosis

• CNS tuberculosis

  • tuberculous rhombencephalitis

• Lyme disease

• neurosyphilis

  • tabes dorsalis

• Rocky Mountain spotted fever

Fungal infections

• CNS aspergillosis

• CNS coccidioidomycosis

• CNS cryptococcosis

• CNS mucormycosis

• neurocandidiasis

Parasitic infections​

• cerebral amoebiasis

  • primary amoebic meningoencephalitis

  • granulomatous amoebic encephalitis

• cerebral malaria

• cerebral sparganosis

• neurocysticercosis

• neurohydatidosis

  • cerebral hydatid disease

  • spinal hydatid disease

• neurotoxoplasmosis

Viral infections

• Eastern equine encephalitis

• enterovirus rhombencephalitis

• flavivirus encephalitis

  • Japanese encephalitis

  • West Nile fever

  • Murray Valley encephalitis

  • St. Louis encephalitis

• herpes virus family

  • herpes simplex virus 1 (HSV-1) encephalitis

  • herpes simplex virus 2 (HSV-2) encephalitis

    • neonatal herpes simplex encephalitis

  • varicella zoster virus (VZV) encephalitis

    • Bickerstaff encephalitis

  • Epstein-Barr virus (EBV) encephalitis

  • cytomegalovirus (CMV) encephalitis

  • human herpesvirus 6 (HHV-6) encephalitis

• HIV CNS manifestations

  • HIV associated neurocognitive disorders (HANDs)

    • HIV-associated dementia

• JC virus

  • progressive multifocal leukoencephalopathy

• measles encephalitis

  • subacute sclerosing panencephalitis

• Nipah virus (NiV) encephalitis

• rabies encephalitis

Prion infections

• Creutzfeldt-Jakob disease (sporadic, variant, familial and iatrogenic)

• fatal familial insomnia

• Gerstmann-Straussler-Scheinker disease

• kuru

• variably protease sensitive prionopathy

AIDS imaging and therapy effects

• HIV/AIDS

CNS trauma

• skull fractures

• perforating injuries

• epidural hematoma

• acute subdural hematoma

• chronic subdural hematoma

• traumatic subarachnoid hemorrhage

• brain contusion

• brain edema

• diffuse axonal injuries

• hemorrhage dating with MRI and CT

• post-traumatic residues

Neurodegenerative diseases

Synucleinopathies

• diseases with Lewy bodies

  • Parkinson disease

    • Parkinson-plus syndrome

  • Lewy body disease

• multiple systemic atrophy (MSA)

  • Shy-Drager syndrome

  • MSA-P (striatonigral degeneration)

  • olivopontocerebellar atrophy (MSA-C)

Tauopathies

• Alzheimer disease

  • typical/classical Alzheimer disease

  • variant (e.g. posterior cortical atrophy)

• chronic traumatic encephalopathy (CTE)

• corticobasal degeneration

• frontotemporal lobar degeneration (FTLD) (not all are tau) 

  • behavioral variant frontotemporal dementia (bvFTLD)

  • language variant frontotemporal dementia (lvFTLD) (primary progressive aphasia (PPA))

    • progressive non-fluent aphasia (PNFA)

    • semantic dementia

    • logopenic dementia

  • right temporal variant frontotemporal dementia

• Pick disease

• progressive supranuclear palsy (PSP)

Cerebral amyloidosis

• cerebral amyloid angiopathy (CAA)

  • Boston criteria for cerebral amyloid angiopathy

  • modified Boston criteria for cerebral amyloid angiopathy

• transthyretin-associated cerebral amyloidosis

• neuronal intranuclear hyaline inclusion disease (NIHID)

Others

• spinocerebellar ataxias

• Huntington disease

• hereditary spastic paraplegia

• amyotrophic lateral sclerosis (ALS)

• clinically unclassifiable parkinsonism (CUP)

• Unverricht-Lundborg disease