Disease Information for Arnold-Chiari syndrome

Ads
Clinical Manifestations
Signs & Symptoms
Anesthetic hypesthetic limbs/areas
Ataxia
Cerebellar signs
Difficulty flexing neck
Dizziness/Dizzy
Dysdiadochokinesia/Alternating failure
Headache in children
Mental Deficiency Child
Nystagmus
Papilledema on exam
Paralysis
Prominent occiput/infant
Pyramidal tract signs
Spastic paralysis/signs
Spastic paraplegia/signs
Staggering Gait
Unable to tandem walk/straight line
Vertical nystagmus
Vertigo
Cervical kyphosis sign
Macrocephaly/Large head
Megalocephaly
Near death experience/infant/SIDS survivor
True Vertigo Sign Confirmed
Vertigo in Children
Disease Progression
Course/Chronic disorder
Course/Chronic only
Demographics & Risk Factors
Population Group
Child
Population/Pediatrics population
Sex & Age Groups
Population/Child
Population/Child-Infant Only
Population/Children/all
Diagnostic Test Results
Other Tests & Procedures
Lumbar puncture/Increased CSF pressure/LP test
CT Scan
MRI/Head cerebellar tonsils/medulla herniated out FM
MRI/MRA/Head neck abnormal
MRI/Neck abnormal
X-RAY
Xray/Cervical spine diagnostic
Xray/Cervical spine abnormal
Xray/Occiput/upper cervical spine indentation
Associated Diseases & Rule outs
Associated Disease & Complications
Arnold-Chiari syndrome
Cervical myelopathy
Charcot neuroarthropathic joint
Congenital Nystagmus
Corpus callosum defect/agenesis
Esotropia
Herniation/Brainstem/peduncles/cerebellar tonsils
Hydrocephalus
Hydrocephalus, acute acquired
Meningoencephalocele
Meningomyelocele
Mental retardation
Paraplegia
Platybasia
Polymicrogyria/congenital
Ptussive Cough Headache Syndrome
Syringobulbia
Syringomyelia
Vocal cord paralysis
Ataxia Disorder
Disease Mechanism & Classification
Class
CLASS/Axial skeletal involvement/disorder (ex)
CLASS/Foramen magnum/skull base defect (ex)
CLASS/Skeletal (category)
Pathophysiology
Pathophysiology/Obstruction fourth ventricle/CNS
Pathophysiology/Congenital CNS/Posterior fossa disorders
Pathophysiology/CSF Pressure Increased
Pathophysiology/Defect foramen magnum/cervical spine defect
Process
PROCESS/Congenital/developmental (category)
PROCESS/Eponymic (category)
PROCESS/Anomalies/Deformities/Malformations (EX)
PROCESS/Congenital CNS disorder (ex)
Synonyms
Synonym
ACM Arnold Chiari malformat, ACM Arnold Chiari malformation, Arnold Chiari syndrome, Arnold Chiari, Arnold Chiari Deformity, ARNOLD CHIARI MALFORMATION, Arnold Chiari obstruction, Arnold Chiari Syndrome, Arnold Chiari syndrome (disorder), CEREBELLOMEDULLARY MALFORMATION SYNDROME, Chiari malformation, Chiari malformation (disorder), Chiari's malformation, Deformity Arnold Chiari, Malformation Arnold Chiari, obstruction Arnold Chiari, Syndrome Arnold Chiari, Synonym/Cerebellomedullary malformation syndrome
Definition

A group of congenital malformations involving the brainstem, cerebellum, upper spinal cord, and surrounding bony structures. Type II is the most common, and features compression of the medulla and cerebellar tonsils into the upper cervical spinal canal and an associated MENINGOMYELOCELE. Type I features similar, but less severe malformations and is without an associated meningomyelocele. Type III has the features of type II with an additional herniation of the entire cerebellum through the bony defect involving the foramen magnum, forming an ENCEPHALOCELE. Type IV is a form a cerebellar hypoplasia. Clinical manifestations of types I-III include TORTICOLLIS; opisthotonus; HEADACHE; VERTIGO; VOCAL CORD PARALYSIS; APNEA; NYSTAGMUS; swallowing difficulties; and ATAXIA. (From Menkes, Textbook of Child Neurology, 5th ed, p261; Davis, Textbook of Neuropathology, 2nd ed, pp236-46)

------------------------------------------

Arnold-Chiari Malformation; ACM; Arnold-Chiari Malformation; Cerebellomedullary Malformation Syndrome

Disorder Subdivisions:Chiari Type I (Chiari Malformation I);Chiari Type II (Chiari Malformation II).

Discussion:Arnold-Chiari malformation is a rare malformation of the brain that is sometimes, but not always, apparent at birth; It is characterized by abnormalities in the area where the brain and spinal cord meet that cause part of the cerebellum to protrude through the bottom of the skull (foramen magnum) into the spinal canal; This interferes with the flow of cerebral spinal fluid to and from the brain, leading to accumulation of cerebral spinal fluid in the empty spaces of the spine and brain; The portion of the cerebellum that protrudes into the spinal canal can become elongated and is called the "cerebellar tonsils" because it resembles the tonsils;

There are four types of Chiari malformation. Depending on the type, the impact on the affected individual may range from mild to severe[NORD 2005]

(Edit)

External Links Related to Arnold-Chiari syndrome
Google
Wikipedia
Merck
Images
PubMed (National Library of Medicine)
NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
Ads