Chiari malformation

Arnold-Chiari malformationArnold–Chiari malformationChiari I malformationChiari malformationsArnold-Chiari syndromeChiariChiari malformation type IArnold Chiari diseaseArnold Chiari II malformationArnold-Chiari malformations
Chiari malformation (CM) is a structural defect in the cerebellum, characterized by a downward displacement of one or both cerebellar tonsils through the foramen magnum (the opening at the base of the skull).wikipedia
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Central sleep apnea

central sleep apnea syndromecentralCentral Apnea
Less often, people may experience ringing or buzzing in the ears, weakness, slow heart rhythm, or fast heart rhythm, curvature of the spine (scoliosis) related to spinal cord impairment, abnormal breathing, such as central sleep apnea, characterized by periods of breathing cessation during sleep, and, in severe cases, paralysis.
Central sleep apnea can also be an indicator of Arnold–Chiari malformation.

Scoliosis

curvature of the spinethoracoplastyidiopathic scoliosis
Less often, people may experience ringing or buzzing in the ears, weakness, slow heart rhythm, or fast heart rhythm, curvature of the spine (scoliosis) related to spinal cord impairment, abnormal breathing, such as central sleep apnea, characterized by periods of breathing cessation during sleep, and, in severe cases, paralysis.
Scoliosis can be associated with amniotic band syndrome, Arnold–Chiari malformation, Charcot–Marie–Tooth disease, cerebral palsy, congenital diaphragmatic hernia, connective tissue disorders, muscular dystrophy, familial dysautonomia, CHARGE syndrome, Ehlers–Danlos syndrome (hyperflexibility, "floppy baby" syndrome, and other variants of the condition), fragile X syndrome, Friedreich's ataxia, hemihypertrophy, Loeys-Dietz syndrome, Marfan's syndrome, nail–patella syndrome, neurofibromatosis, osteogenesis imperfecta, Prader–Willi syndrome, proteus syndrome, spina bifida, spinal muscular atrophy, syringomyelia, and pectus carinatum.

Hydrocephalus

hydrocephalyhydrocephalicobstructive hydrocephalus
This can sometimes lead to non-communicating hydrocephalus as a result of obstruction of cerebrospinal fluid (CSF) outflow.
Other causes of congenital hydrocephalus include neural-tube defects, arachnoid cysts, Dandy-Walker syndrome, and Arnold-Chiari malformation.

Syringomyelia

a crippling illnessa spine disorder
The blockage of cerebrospinal fluid (CSF) flow may also cause a syrinx to form, eventually leading to syringomyelia. Other conditions sometimes associated with Chiari malformation include hydrocephalus, syringomyelia, spinal curvature, tethered spinal cord syndrome, and connective tissue disorders such as Ehlers-Danlos syndrome and Marfan syndrome.
The first major form relates to an abnormality of the brain called an Arnold–Chiari malformation or Chiari Malformation.

Tinnitus

ringing in the earspulsatile tinnitusringing
Less often, people may experience ringing or buzzing in the ears, weakness, slow heart rhythm, or fast heart rhythm, curvature of the spine (scoliosis) related to spinal cord impairment, abnormal breathing, such as central sleep apnea, characterized by periods of breathing cessation during sleep, and, in severe cases, paralysis.

Hans Chiari

ChiariHans von Chiari
The malformation is named for Austrian pathologist Hans Chiari.
The phenomenon was later to become known as the "Arnold-Chiari malformation", named after Chiari and German pathologist, Julius Arnold (1835–1915).

Cerebellar tonsil

cerebellar tonsilsTonsiltonsil of cerebellum
Chiari malformation (CM) is a structural defect in the cerebellum, characterized by a downward displacement of one or both cerebellar tonsils through the foramen magnum (the opening at the base of the skull).
A Type I Chiari malformation is a congenital anomaly of the brain in which the cerebellar tonsils are elongated and pushed down through the opening of the base of the skull (see foramen magnum), blocking the flow of cerebrospinal fluid (CSF) as it exits through the medial and lateral apertures of the fourth ventricle.

Vertigo

dizzinessvertiginousperipheral vertigo
A number of conditions that involve the central nervous system may lead to vertigo including: lesions caused by infarctions or hemorrhage, tumors present in the cerebellopontine angle such as a vestibular schwannoma or cerebellar tumors, epilepsy, cervical spine disorders such as cervical spondylosis, degenerative ataxia disorders, migraine headaches, lateral medullary syndrome, Chiari malformation, multiple sclerosis, parkinsonism, as well as cerebral dysfunction.

Intracranial pressure

increased intracranial pressureintracranial hypertensionpressure inside the skull

Lhermitte's sign

Lhermitte sign
Although often considered a classic finding in multiple sclerosis, it can be caused by a number of conditions, including transverse myelitis, Behçet's disease, trauma, radiation myelopathy, vitamin B12 deficiency (subacute combined degeneration), compression of the spinal cord in the neck from any cause such as cervical spondylosis, disc herniation, tumor, and Arnold-Chiari malformation.

Julius Arnold

A type II CM is also known as an Arnold–Chiari malformation in honor of Chiari and German pathologist Julius Arnold.
With Austrian pathologist, Hans Chiari, his name is lent to a condition known as Arnold–Chiari malformation, a disorder that takes place when the cerebellar tonsils and the medulla oblongata protrude through the foramen magnum into the spinal canal, without displacing the lower brain stem.

Foramen magnum

basionopisthionforamen occipitale magnum
Chiari malformation (CM) is a structural defect in the cerebellum, characterized by a downward displacement of one or both cerebellar tonsils through the foramen magnum (the opening at the base of the skull).

Nystagmus

nystagmus, pathologicPathologic nystagmusHorizontal gaze nystagmus
Nystagmus is often very commonly present with Chiari malformation.

Valsalva maneuver

Valsalva manoeuvreValsalvaany activity that further increases the intracranial pressure
Headache and pain upon performing the Valsalva maneuver is also one of the main symptoms in Arnold–Chiari malformation.

Colpocephaly

It is a nonspecific finding and is associated with multiple neurological syndromes, including agenesis of the corpus callosum, Chiari malformation, lissencephaly, and microcephaly.

Craniosynostosis

craniosynostosescraniostenosisCloverleaf skull
Congenital causes include hydrocephalus, craniosynostosis (especially of the lambdoid suture), hyperostosis (such as craniometaphyseal dysplasia, osteopetrosis, erythroid hyperplasia), X-linked vitamin D-resistant rickets, and neurofibromatosis type I.
This can be further complicated with a possible Arnold-Chiari malformation, which can partially obstruct the flow of cerebro-spinal fluid from the neurocranium to the spinal cord.

Syrinx (medicine)

syrinxsyrinxes
The blockage of cerebrospinal fluid (CSF) flow may also cause a syrinx to form, eventually leading to syringomyelia.
At least ½ of syrinxes occur in patients with congenital abnormalities of the craniocervical junction (e.g. herniation of cerebellar tissue into the spinal canal, called Chiari malformation), brain (e.g. encephalocele), or spinal cord (e.g. myelomeningocele—see Congenital Neurologic Anomalies: Brain Anomalies).

Ehlers–Danlos syndromes

Ehlers-Danlos syndromeEhlers–Danlos syndromeEhlers Danlos Syndrome
Other conditions sometimes associated with Chiari malformation include hydrocephalus, syringomyelia, spinal curvature, tethered spinal cord syndrome, and connective tissue disorders such as Ehlers-Danlos syndrome and Marfan syndrome.

Spina bifida

myelomeningocelemeningocelespina bifida occulta
Myelomeningocele is also associated with Arnold-Chiari malformation, necessitating a VP shunt placement.

Spontaneous cerebrospinal fluid leak

intracranial hypotensionSpontaneous Intracranial HypotensionCSF leaks
In Pseudo-Chiari Malformation, leaking of CSF may cause displacement of the cerebellar tonsils and similar symptoms sufficient to be mistaken for a Chiari I malformation.
Low CSF volume can cause the cerebellar tonsil position to descend, which can be mistaken for Chiari malformation; however when the CSF leak is repaired the tonsil position often returns to normal (as seen in upright MRI) in this "pseudo-Chiari" condition.

Posterior cranial fossa

posterior fossacranial fossa, posteriorcranial cavity
The most widely accepted pathophysiological mechanism by which Chiari type I malformations occur is by a reduction or lack of development of the posterior fossa as a result of congenital or acquired disorders.
An underdeveloped posterior cranial fossa can cause Arnold-Chiari Malformation.

J. B. Holmes

JB HolmesJohn B. Holmes
He had been dealing with vertigo symptoms for several months and eventually was diagnosed with structural defects in the cerebellum known as Chiari malformations.

The Choice (House)

The ChoiceThe Choice" (''House'')
Chiari malformation was briefly mentioned on the medical drama House M.D. in the fifth season episode "House Divided", and it was the focus of the sixth season episode "The Choice."
Teddy has an Arnold-Chiari malformation, a narrowing in the base of his skull.