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Disorder of the central nervous system

MedGen UID:: 3306
•Concept ID:: C0007682
•: Disease or Syndrome

Synonyms:	Central Nervous System Disease; Central Nervous System Diseases; Central Nervous System Disorder; Central Nervous System Disorders; CNS Disease; CNS Diseases
SNOMED CT:	Disorder of the central nervous system (23853001); Central nervous system (CNS) disease (23853001); Encephalomyeloneuropathy (23853001)

Definition

A non-neoplastic or neoplastic disorder which affects the brain and/or spinal cord. [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVDisorder of the central nervous system

Pathological Conditions, Signs and Symptoms
- Pathological process
  - Disease
    - Disorder by Site
      - Disorder of nervous system
        Disorder of the central nervous system
        Brain disorder
        Acute Febrile Encephalopathy
        Akinetic mutism
        Amblyopia
        Brain abscess
        Cerebral toxoplasmosis
        Brain Death
        Central auditory dysfunction
        Auditory processing disorder
        Central hearing loss
        Cerebellar disorder
        Cerebellar ataxia
        Cerebellar neoplasm
        Dandy-Walker syndrome
        Miller Fisher syndrome
        Paraneoplastic cerebellar degeneration
        spinocerebellar disease
        Cerebrovascular disorder
        Arteriovenous Malformation, Intracranial
        Basal ganglia cerebrovascular disorder
        Carotid artery disorder
        Central nervous system vasculitis
        Cerebral amyloid angiopathy
        Cerebral infarction
        Cerebral ischemia
        Cerebral Vascular Insufficiency
        Intracranial arterial disease
        Intracranial Embolism and Thrombosis
        Intracranial hemorrhage
        Intracranial vasospasm
        Ischemic encephalopathy
        Periventricular leukomalacia
        Small vessel cerebrovascular disease
        Sneddon syndrome
        Stroke disorder
        Susac syndrome
        Vascular dementia
        Vascular Depression
        Vascular headache
        Chronic brain syndrome
        Cerebral palsy
        Persistent vegetative state
        Dementia
        Alzheimer disease
        Creutzfeldt-Jakob disease
        Degenerations, Frontotemporal Lobar
        Dementia associated with AIDS
        Diffuse Neurofibrillary Tangles with Calcification
        Frontal lobe dementia
        Frontolimbic dementia
        Frontotemporal dementia
        Huntington disease
        Kluver-Bucy syndrome
        Lewy body dementia
        Presenile dementia
        Primary progressive aphasia
        Senile dementia
        Subcortical dementia
        Diaschisis
        Disorder of basal ganglia
        Blepharospasm-oromandibular dystonia syndrome
        Chorea gravidarum
        Multiple system atrophy
        Neuroleptic malignant syndrome
        Parkinsonian disorder
        Pigmentary pallidal degeneration
        Progressive supranuclear palsy
        Torsion dystonia
        Tourette syndrome
        Wilson disease
        Encephalitis
        Anti-NMDA receptor encephalitis
        Infectious encephalitis
        Limbic encephalitis
        Meningoencephalitis
        Pallor
        Rasmussen subacute encephalitis
        Steroid-responsive encephalopathy associated with autoimmune thyroiditis
        Encephalomalacia
        Encephalopathy with sepsis
        Epilepsy
        Absence seizure
        Aggravated Epilepsy
        Anosognostic Epilepsy
        Atonic epileptic seizure
        Benign Familial Convulsion
        Benign Neonatal Epilepsy
        Benign Rolandic epilepsy
        Congenital Epilepsy
        Epilepsy syndrome
        Epilepsy with generalized tonic-clonic seizures
        Extratemporal epilepsy
        Focal epilepsy
        Generalized epilepsy
        Juvenile myoclonic epilepsy
        Lennox-Gastaut syndrome
        Post-traumatic epilepsy
        Progressive myoclonic epilepsy
        Reflex epilepsy
        Refractory epilepsy
        Severe myoclonic epilepsy in infancy
        Sudden unexpected death in epilepsy
        Headache disorder
        Headache Disorders, Primary
        Headache Disorders, Secondary
        Hydrocephalus
        Communicating hydrocephalus
        Congenital hydrocephalus
        External hydrocephalus
        Noncommunicating hydrocephalus
        Normal pressure hydrocephalus
        Post-Inflammatory Hydrocephalus
        Posthemorrhagic hydrocephalus
        Severe hydrocephalus
        Hypothalamic disorder
        Bardet-Biedl syndrome
        Hypothalamic neoplasm
        Laurence-Moon syndrome
        Pituitary gland disorder
        Hypoxia of brain
        Brain hypoxia - ischemia
        Increased intracranial pressure
        Benign intracranial hypertension
        Hypertensive encephalopathy
        Intracranial hypotension
        Intracranial swelling
        Leukoencephalopathy
        CNS demyelinating autoimmune disease
        Diffuse leukoencephalopathy
        Diffuse spongiform leukoencephalopathy
        Hereditary Central Nervous System Demyelinating Diseases
        Hereditary diffuse leukoencephalopathy with spheroids
        Human immunodeficiency virus leukoencephalopathy
        Posterior leukoencephalopathy
        Posterior leukoencephalopathy syndrome
        Posterior reversible encephalopathy syndrome
        Progressive leukoencephalopathy
        Progressive multifocal leukoencephalopathy
        Vanishing white matter disease
        Metabolic encephalopathy
        Bilirubin encephalopathy
        Brain Diseases, Metabolic, Inborn
        Central pontine myelinolysis
        Hepatic encephalopathy
        Marchiafava-Bignami disease
        Mitochondrial encephalomyopathy
        Reye syndrome
        Wernicke encephalopathy
        Neoplasm of brain
        Adult Brain Neoplasm
        Benign neoplasm of brain
        Cerebral ventricle cancer
        Childhood Brain Neoplasm
        Infratentorial neoplasm
        Malignant neoplasm of brain
        Neurocytoma
        Pinealoma
        Primary brain neoplasm
        Supratentorial neoplasm
        Neuroaxonal dystrophy
        Non-Neoplastic Brain Disorder
        Brain Development Abnormality
        Cerebellar degeneration
        Cerebellar hypoplasia
        Cerebral abscess
        Cerebritis
        Corticobasal degeneration disorder
        Encephalopathy
        Hamartoma of brain
        Hemimegalencephaly
        Olivopontocerebellar atrophy
        Schizencephaly
        Thalamic disorder
        Organic mental disorder
        Nonpsychotic Mental Disorder Following Organic Brain Damage
        Transient organic mental disorder
        Schilder disease
        Transient global amnesia
        High pressure neurological syndrome
        Hyperekplexia
        Developmental and epileptic encephalopathy, 8
        Hereditary hyperekplexia
        Hyperekplexia 1
        Hyperekplexia 2
        Hyperekplexia 3
        Hyperekplexia 4
        Sporadic hyperekplexia
        Infectious disease of central nervous system
        Arachnoiditis
        Spinal arachnoiditis
        Bacterial infection of central nervous system
        Bacterial meningitis
        Central nervous system tuberculosis
        Lyme neuroborreliosis
        Neurosyphilis
        Encephalomyelitis
        Acute disseminated encephalomyelitis
        Encephalomyelitis, Equine
        Infection causing encephalomyelitis
        Myalgic encephalomeyelitis/chronic fatigue syndrome
        Epidural abscess
        Fungal infection of central nervous system
        Fungal meningitis
        Neuroaspergillosis
        HTLV-1-associated myelopathy-tropical spastic paraparesis
        Infectious meningitis
        Cryptococcal meningitis
        Viral meningitis
        Infectious Meningoencephalitis
        Myelitis
        Acute flaccid myelitis
        Acute transverse myelitis
        Poliomyelitis
        Transverse myelitis
        Parasitic infection of central nervous system
        Central Nervous System Helminthiasis
        Central Nervous System Protozoal Infections
        Perimeningeal Infections
        Subdural empyema
        Prion disease
        Bovine Spongiform Encephalopathy
        Fatal familial insomnia
        Gerstmann-Straussler-Scheinker syndrome
        Kuru
        Scrapie
        Variant Creutzfeldt-Jakob disease
        Wasting Disease, Chronic
        Viral infection of central nervous system
        Pseudorabies
        Viral encephalitis
        Movement disorder
        Abnormal head movements
        Jerky head movements
        Abnormal posturing
        Abnormal reflex
        Abnormal postural reflex
        Abnormal superficial reflex
        Hyperreflexia
        Primitive reflex
        Reduced tendon reflexes
        Angelman syndrome
        Angelman syndrome due to a point mutation
        Angelman syndrome due to imprinting defect in 15q11-q13
        Angelman syndrome due to maternal 15q11q13 deletion
        Angelman syndrome due to paternal uniparental disomy of chromosome 15
        Apraxia
        Alien Hand Syndrome
        Gait apraxia
        Hand apraxia
        Ideomotor apraxia
        Limb apraxia
        Oculomotor apraxia
        Oromotor apraxia
        Speech apraxia
        Astasia
        Asterixis
        Chorea
        Chorea-acanthocytosis
        Sydenham chorea
        Chronic tic disorder
        Diminished movement
        Akinesia
        Hypokinesia
        Drug-induced akathisia
        Dyskinesia
        Athetosis
        Catalepsy
        Drug-induced dyskinesia
        Dyssynergia
        Dystonic disorder
        Extrapyramidal dyskinesia
        Habit tic
        Hyperkinetic movements
        Myoclonus
        Orofacial dyskinesia
        psychomotor agitation
        Synkinesis
        Tardive dyskinesia
        Tremor
        Dystonia
        Dystonia 12
        Dystonia 16
        Episodic kinesigenic dyskinesia 1
        Familial Dystonia
        Essential tremor
        Tremor, hereditary essential, 1
        Tremor, hereditary essential, 2
        Tremor, hereditary essential, 3
        Tremor, hereditary essential, and idiopathic normal pressure hydrocephalus
        Frontal release signs
        Gait disturbance
        Antalgic gait
        Broad-based gait
        Cautious gait
        Cerebellar ataxia associated with quadrupedal gait
        Crouch gait
        Difficulty walking backward
        Dystonic gait
        Falls
        Freezing of gait
        Gait ataxia
        Gait imbalance
        Impaired heel-walking ability
        Impaired tandem gait
        Impaired toe-walking ability
        Inability to walk
        Intoeing
        Neurogenic claudication
        Out-toeing
        Shuffling gait
        Spastic gait
        Steppage gait
        Tip-toe gait
        Unsteady gait
        Waddling gait
        Involuntary movements
        Akathisia
        Alien limb phenomenon
        Chvostek sign
        Clonus
        Fasciculations
        Hemiballismus
        Paroxysmal dyskinesia
        Self-mutilation of tongue and lips due to involuntary movements
        Shakes
        Muscle fibrillation
        Facial-lingual fasciculations
        Myokymia
        Eyelid myokymia
        Facial myokymia
        Postural instability
        Stooped posture
        Tic
        Tourettism
        Transient tic disorder
        X-linked dystonia-parkinsonism
        Myelopathy
        Amyotrophic lateral sclerosis
        Amyotrophic Lateral Sclerosis 14, With or Without Frontotemporal Dementia
        Amyotrophic lateral sclerosis type 1
        Amyotrophic lateral sclerosis type 3
        Amyotrophic lateral sclerosis type 4
        Amyotrophic lateral sclerosis type 6
        Amyotrophic lateral sclerosis type 7
        Amyotrophic lateral sclerosis type 8
        Amyotrophic lateral sclerosis type 9
        Amyotrophic lateral sclerosis type 10
        Amyotrophic lateral sclerosis type 11
        Amyotrophic lateral sclerosis type 21
        Amyotrophic lateral sclerosis type 23
        Juvenile amyotrophic lateral sclerosis
        Progressive muscular atrophy
        Cervical myelopathy
        Abnormal cervical myelogram
        Hydromyelia
        Infarction of spinal cord
        Spinal Cord Infarct During Birth
        Myelocystocele
        Pneumorrhachis
        Spinal cord compression
        Cervical cord compression
        Spinal Cord Hamartoma
        Spinal cord tumor
        Adult Spinal Cord Neoplasm
        Benign neoplasm of spinal cord
        Cervicomedullary junction neoplasm
        Childhood spinal cord tumor
        Conus medullaris neoplasm
        Epidural spinal canal neoplasm
        Recurrent Spinal Cord Neoplasm
        Refractory Spinal Cord Neoplasm
        Spinal cord cancer
        Spinal cord glioma
        Spinal cord intramedullary teratoma
        Spinal meningioma
        Spinal Cord Vascular Diseases
        Spinal cord ischemia
        Spinal muscular atrophy
        Acute infantile spinal muscular atrophy
        Kennedy disease
        Kugelberg-Welander disease
        Progressive spinal muscular atrophy
        Proximal Hereditary Motor Neuropathy Type I
        Proximal spinal muscular atrophy
        Spinal Muscular Atrophy of Childhood
        Spinal muscular atrophy, segmental
        Spinal muscular atrophy, type II
        Spinal muscular atrophy, type IV
        Werdnig-Hoffmann disease
        Stiff-man syndrome
        Classic stiff person syndrome
        Focal stiff limb syndrome
        Progressive encephalomyelitis with rigidity and myoclonus
        Subacute combined degeneration of spinal cord
        Syringomyelia
        Primary syringomyelia
        Secondary syringomyelia
        Syringomyelia and Hydromyelia
        Tabes dorsalis
        Neoplasm of the central nervous system
        Adult Central Nervous System Neoplasm
        Adult astrocytic tumor
        Adult central nervous system germ cell tumor
        Adult central nervous system primitive neuroectodermal neoplasm
        Adult embryonal tumor with multilayered rosettes, c19mc-altered
        Adult Ependymal Tumor
        Adult Ganglioglioma
        Adult Intracranial Neoplasm
        Adult Meningioma
        Adult Oligodendroglial Tumor
        Benign neoplasm of the central nervous system
        Cerebral hamartoma
        Choroid plexus papilloma
        Hemangioblastoma
        Meningioma
        Multiple central nervous system lipomas
        Neuroepithelial cyst
        Central nervous system cyst
        Arachnoid cyst
        Central Nervous System Colloid Cyst
        Central Nervous System Epidermoid Cyst
        Cerebral cyst
        Choroid plexus cyst
        Colloid cyst
        Endodermal Cyst
        Ependymal cyst
        Intracranial Colloid Cyst
        Intracranial cystic lesion
        Intraspinal Cyst
        Meningeal cyst
        Neurenteric cyst
        Perineural cyst
        Pineal cyst
        Pituitary gland cyst
        Rathke pouch cyst
        Central nervous system hematopoietic neoplasm
        Central Nervous System Histiocytic and Dendritic Cell Neoplasm
        Central nervous system leukemia
        Intracranial Myeloid Sarcoma
        Primary central nervous system lymphoma
        Secondary Central Nervous System Lymphoma
        Childhood Central Nervous System Neoplasm
        Childhood astrocytic tumor
        Childhood Benign Central Nervous System Neoplasm
        Childhood central nervous system germ cell tumor
        Childhood Ependymal Tumor
        Childhood ganglioglioma
        Childhood Intracranial Neoplasm
        Childhood Malignant Central Nervous System Neoplasm
        Childhood Mixed Glioma
        Childhood oligodendroglioma
        Pediatric meningioma
        Recurrent Childhood Central Nervous System Neoplasm
        Intracranial Neoplasm
        Benign Intracranial Neoplasm
        Intracranial meningioma
        Malignant Intracranial Neoplasm
        Metastatic Intracranial Malignant Neoplasm
        Posterior Fossa Neoplasm
        Recurrent Intracranial Neoplasm
        Skull base neoplasm
        Visual Pathway Neoplasm
        Intraspinal Neoplasm
        Cauda equina neoplasm
        Intradural extramedullary spinal canal neoplasm
        Intraspinal meningioma
        Malignant neoplasm of the central nervous system
        Anaplastic ganglioglioma
        Central Nervous System Carcinoma
        Central Nervous System Embryonal Tumor
        Central Nervous System Histiocytic Sarcoma
        Central Nervous System Melanoma
        Cerebral germinoma
        Choroid plexus carcinoma
        Glioma
        Malignant Central Nervous System Germ Cell Tumor
        Malignant central nervous system mesenchymal, non-meningothelial neoplasm
        Malignant glioma
        Malignant tumor of meninges
        Medulloblastoma
        Metastatic Malignant Neoplasm in the Central Nervous System
        Neuroblastic tumor
        Recurrent Primary Malignant Central Nervous System Neoplasm
        Refractory Primary Malignant Central Nervous System Neoplasm
        Retinoblastoma
        Neuronal/glioneuronal neoplasm of the central nervous system
        Central neurocytoma
        Cerebellar Glioneuronal and Neuronal Tumors
        Desmoplastic infantile astrocytoma
        Desmoplastic infantile astrocytoma/ganglioglioma
        Desmoplastic infantile ganglioglioma
        Diffuse leptomeningeal glioneuronal tumor
        Dysembryoplastic neuroepithelial tumor
        Dysembryoplastic Neuroepithelial-Like Tumor of the Septum Pellucidum
        Extraventricular neurocytoma
        Gangliocytoma
        Ganglioglioma
        Ganglioneuroma
        Lhermitte-Duclos disease
        Myxoid glioneuronal tumor
        Papillary glioneuronal tumor
        Polymorphous low grade neuroepithelial tumor of the young
        Rosette-forming glioneuronal tumor
        Primary Central Nervous System Neoplasm
        Benign neoplasm of meninges
        Central nervous system germ cell tumor
        Central Nervous System Hemangioblastoma
        Central nervous system melanocytic neoplasm
        Central nervous system mesenchymal non-meningothelial tumor
        Central Nervous System Paraganglioma
        Central Nervous System SMARCB1/INI1-Deficient Tumor
        Metastatic Primary Malignant Central Nervous System Neoplasm
        Neoplasm of the posterior pituitary
        Recurrent Primary Central Nervous System Neoplasm
        Refractory Primary Central Nervous System Neoplasm
        Sellar Chordoma
        Sellar Neuroblastoma
        Tumor of meninges
        Familial multiple meningioma
        Leptomeningeal Neoplasm
        Meningeal Carcinomatosis
        Meninges hemangiopericytoma
        Primary melanocytic lesion of meninges
        Non-Neoplastic Central Nervous System Disorder
        Alice in Wonderland syndrome
        Central Nervous System Hamartoma
        Degenerative disease of the central nervous system
        Brachial amyotrophic diplegia
        Demyelinating disease of central nervous system
        Dentatorubral-pallidoluysian atrophy
        Extrapyramidal disease
        Frontotemporal dementia with motor neuron disease
        Hereditary Neurodegenerative Disorder
        Hereditary spastic paraplegia
        Pelizaeus-Merzbacher disease
        Pick disease
        Spinocerebellar atrophy
        Striatonigral degeneration
        Infantile neuroaxonal dystrophy
        Meningitis
        Aseptic meningitis
        Chronic lymphocytic meningitis
        Eosinophilic meningitis
        Lymphocytic choriomeningitis
        Multiple sclerosis
        Chronic progressive multiple sclerosis
        Relapsing remitting multiple sclerosis
        Neurodevelopmental disorder
        Attention Deficit and Disruptive Behavior Disorders
        Child Behavior Disorders
        Child Development Disorders, Pervasive
        Childhood-onset schizophrenia
        communication disorder
        Congenital meningocele
        Developmental disorder
        Fetal Neurodevelopmental Disorder
        Gabriele de Vries syndrome
        Intellectual disability
        Learning disability
        Motor skill disorder
        Mutism
        Myelocele
        reactive attachment disorder
        Separation anxiety disorder
        Stereotypic movement disorder
        Neuronitis
        Polyradiculopathy
        Secondary Parkinson disease
        Postencephalitic Parkinson disease
        Sensory disorder
        Disorder of taste
        Tactile Disorder
        Spina bifida
        Cervical spina bifida
        Myelomeningocele
        Spina bifida cystica
        Spina bifida occulta
        Vascular malformation of the nervous system
        Central Nervous System Cavernous Hemangioma
        Central Nervous System Venous Angioma
        Cerebral arteriovenous malformation
        Cerebral cavernous malformation
        Meningioangiomatosis
        Sinus pericranii
        Vein of Galen malformation
        Ocular motility disease
        Duane retraction syndrome
        Duane retraction syndrome 2
        Duane syndrome type 1
        Nystagmus
        Congenital nystagmus
        Convergence-retraction nystagmus
        Divergence nystagmus
        Gaze-evoked nystagmus
        Horizontal nystagmus
        Nystagmus-induced head nodding
        Pendular nystagmus
        Rotary nystagmus
        Vertical nystagmus
        Oculomotor nerve palsy
        Congenital oculomotor nerve palsy
        Holmes-Adie syndrome
        Ophthalmoplegia
        External ophthalmoplegia
        Internal ophthalmoplegia
        Internuclear ophthalmoplegia
        Progressive external ophthalmoplegia
        Progressive ophthalmoplegia
        Recurrent painful ophthalmoplegic neuropathy
        Supranuclear ophthalmoplegia
        Total ophthalmoplegia
        Opsoclonus-myoclonus syndrome
        Strabismus
        Concomitant strabismus
        Cyclodeviation
        Esodeviation
        Esotropia
        Exodeviation
        Exotropia
        Heterophoria
        Heterotropia
        Hyperdeviation
        Hypertropia
        Hypodeviation
        Hypotropia
        Incomitant strabismus
        Monocular strabismus
        Paralytic strabismus
        Tolosa-Hunt syndrome
        Pneumocephalus

Professional guidelines

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Differential diagnosis of multiple sclerosis and other inflammatory CNS diseases.

Wildner P, Stasiołek M, Matysiak M
Mult Scler Relat Disord 2020 Jan;37:101452. Epub 2019 Oct 15 doi: 10.1016/j.msard.2019.101452. PMID: 31670010

Alexander disease.

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Acuted disseminated encephalomyelitis.

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Acute disseminated encephalomyelitis.

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Clinical prediction guides

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Huntington's disease.

Craufurd D
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Recent systematic reviews

The Role of Selected Interleukins in the Development and Progression of Multiple Sclerosis-A Systematic Review.

Grunwald C, Krętowska-Grunwald A, Adamska-Patruno E, Kochanowicz J, Kułakowska A, Chorąży M
Int J Mol Sci 2024 Feb 23;25(5) doi: 10.3390/ijms25052589. PMID: 38473835 Free PMC Article

Clinical characteristics, management, and outcomes of CLIPPERS: A comprehensive systematic review of 140 patients from 100 studies.

Al-Chalabi M, DelCimmuto NR, Beran A, Devarasetty PP, Mhanna A, Mahfooz N, Sheikh A
Mult Scler Relat Disord 2022 Dec;68:104112. Epub 2022 Aug 18 doi: 10.1016/j.msard.2022.104112. PMID: 36029706

Laquinimod for multiple sclerosis.

He D, Han K, Gao X, Dong S, Chu L, Feng Z, Wu S
Cochrane Database Syst Rev 2013 Aug 6;2013(8):CD010475. doi: 10.1002/14651858.CD010475.pub2. PMID: 23922214 Free PMC Article

Antidepressant drugs for narcolepsy.

Vignatelli L, D'Alessandro R, Candelise L
Cochrane Database Syst Rev 2008 Jan 23;2008(1):CD003724. doi: 10.1002/14651858.CD003724.pub3. PMID: 18254030 Free PMC Article

Antidepressant drugs for narcolepsy.

Vignatelli L, D'Alessandro R, Candelise L
Cochrane Database Syst Rev 2005 Jul 20;(3):CD003724. doi: 10.1002/14651858.CD003724.pub2. PMID: 16034909

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