Abstract A case review of a healthy 3-year-old toddler with a chief complaint of sleep-disordered breathing is presented. The child underwent a pneumogram sleep study with significant abnormal findings. [ncbi.nlm.nih.gov]

Clinical presentation of type 2 Chiari malformation. The presentation depend on the age of onset. If present during early infancy, usually the key features is of brainstem compression. [teddybrain.wordpress.com]

Presentation Chiari type I [ 5 ] The presentation of this condition depends upon the severity of the herniation and associated other features. [patient.info]

This patient presented with symptoms induced by a cervical lesion at 65 years of age. [nature.com]

• Respiratory Insufficiency

  insufficiency (29%), cranial nerve paralysis (17%) and cardiac arrest (14.5%) among the most frequent manifestations. [raredr.com]

• Orthostatic Hypotension

  We report the successful management of a patient of syringomyelia with ACM who developed orthostatic hypotension during positioning for foramen magnum decompression. [apicareonline.com]

• Neck Pain

  Symptoms, which may not appear until late childhood or adulthood, include severe headache, neck pain, imbalance, dizziness, swallowing problems, numbness in the hands, depression, fatigue, and sleep problems. [mayfieldchiaricenter.com]

  General Chiari Malformation Symptoms The Chiari malformation without syringomyelia can cause neck pain and occipital (base of the skull) headaches with coughing, exertion or sneezing. [neckandback.com]

  If you have symptoms, they may include Neck pain Balance problems Numbness or other abnormal feelings in the arms or legs Dizziness Vision problems Difficulty swallowing Poor hand coordination Doctors diagnose CM using imaging tests. [medlineplus.gov]

  Symptoms (important to remember that many of these are from birth) headache pain at the base of the skull (often worse with exertion) neck pain dizziness clumsiness/imbalance sudden drop attacks (buckling of legs) swallowing problems visual changes spasticity [portchiro.com]

• Torticollis

  Clinical manifestations of types I-III include TORTICOLLIS ; opisthotonus; HEADACHE ; VERTIGO ; VOCAL CORD PARALYSIS ; APNEA ; NYSTAGMUS, CONGENITAL ; swallowing difficulties; and ATAXIA. [hon.ch]

• Episodic Headache

  […] sensory disturbances (numbness, pain, and temperature deficits).[ 10 32 ] Often headaches originating from ACM-1 can be confused with other types of headaches; however, the typical description of the headaches in ACM-1 are described by patients as pressure-like [surgicalneurologyint.com]

• Headache

  Curr Pain Headache Rep. 2011;15(3):157-163. [consultant360.com]

  One of the more common reasons for a patient to see a chiropractor is for treatment of headaches. [dynamicchiropractic.com]

  […] sensory disturbances (numbness, pain, and temperature deficits).[ 10 32 ] Often headaches originating from ACM-1 can be confused with other types of headaches; however, the typical description of the headaches in ACM-1 are described by patients as pressure-like [surgicalneurologyint.com]

  Chiari Malformation Symptoms The most common symptom experienced by persons with Chiari malformation is a headache. The headache usually starts at the back of the person's head and neck, then radiates upward. [disabled-world.com]

  Symptoms include headache, neck pain, dizziness, arm numbness or weakness, sleep problems, fatigue, etc. Headache in the back of the head that worsens with coughing, sneezing, or straining is a hallmark symptom. Chiari affects children and adults. [mayfieldchiaricenter.com]

• Nystagmus

  Arnold-Chiari malformation nystagmus nystagmus of skew eye movement recordings Statistics from Altmetric.com Arnold-Chiari malformation nystagmus nystagmus of skew eye movement recordings In the Arnold-Chiari malformation a part of the brainstem and the [jnnp.bmj.com]

  Much stronger downbeating nystagmus seen on eccentric gaze, in patient with a prominent Chiari Malformation. There is no rebound nystagmus -- he has a right-beating nystagmus that remains direction fixed on centering. [dizziness-and-balance.com]

  Patients most likely to benefit from surgery are those with reproducible neurological signs - eg, nystagmus, absent gag reflex, and demonstrably abnormal CSF flow in the hindbrain on cine MRI [ 9 ]. [patient.info]

  Nystagmus (irregular eye movements; typically, so-called "downbeat nystagmus"). Dysautonomia : tachycardia (rapid heart), syncope (fainting), polydipsia (extreme thirst), chronic fatigue. [disabled-world.com]

  Clinical manifestations of types I-III include TORTICOLLIS ; opisthotonus; HEADACHE ; VERTIGO ; VOCAL CORD PARALYSIS ; APNEA ; NYSTAGMUS, CONGENITAL ; swallowing difficulties; and ATAXIA. [hon.ch]

• Vertigo

  Clinical manifestations of types I-III include TORTICOLLIS ; opisthotonus; HEADACHE ; VERTIGO ; VOCAL CORD PARALYSIS ; APNEA ; NYSTAGMUS, CONGENITAL ; swallowing difficulties; and ATAXIA. [hon.ch]

  They may experience vertigo, difficulties with balancing, and dizziness. [disabled-world.com]

• Cerebellar Ataxia

  […] movements when walking 0002066 Increased intracranial pressure Rise in pressure inside skull 0002516 Lower limb hyperreflexia Overactive lower leg reflex 0002395 Myelopathy 0002196 Nystagmus Involuntary, rapid, rhythmic eye movements 0000639 Progressive cerebellar [rarediseases.info.nih.gov]

• Sinus Arrhythmia

  Sinus arrhythmia and pupil size in Chiari I malformation: evidence of autonomic dysfunction. Funct Neurol 1993;8(4):251-7. [PubMed] Nogues M, Delorme R, Saadia D, Heidel K, Benarroch E. [apicareonline.com]

Surgical treatment of Chiari malformation with and without syringomyelia: experience with 177 adult patients. J Neurosurg. 2013;118:232-242. Deng X, Wu L, Yang C, Tong X, Xu Y. Surgical Treatment of Chiari I malformation with ventricular dilation. [raredr.com]

INTRODUCTION: Several different surgical techniques have been used in the treatment of patients with symptomatic Arnold-Chiari malformation type 1 (ACM-1) with or without syrinx. [ncbi.nlm.nih.gov]

Abstract Introduction Several different surgical techniques have been used in the treatment of patients with symptomatic Arnold-Chiari malformation type 1 (ACM-1) with or without syrinx. [thieme-connect.com]

Surgical treatment for syryngomyelia associated with a Chiari malformation. Nippon Saigai Igakukai Kaishi 1995; 43 : 788–797 (in Japanese). 16 Hoffman HJ, Hendrick EB, Humphreys RP. [nature.com]

What Are Some Common Treatments? If a patient is confirmed to have a significant Type I Arnold-Chiari malformation treatment usually depends on symptoms and the patient's preferences. Asymptomatic patients usually do not require treatment. [nervous-system-diseases.com]

Prognosis and Outcomes The key to good prognosis of Chiari II malformation is early recognition of symptoms and early treatment. Patients with less severe symptoms at diagnosis are much more likely to improve with surgical intervention. [csfinfo.org]

The prognosis otherwise depends on severity of the malformation, associated neurological abnormalities and complications. There has been some improvement in prognosis with improved diagnostic and surgical techniques. [patient.info]

[…] malformation: type I: herniation of the cerebellar tonsils associated with syringomyelia type II: herniation of the cerebellar vermis and tonsils associated with meningomyeloceles and hydrocephalus Links: types clinical features investigations treatment prognosis [gpnotebook.co.uk]

Prevention, and Comlications Prognosis range widely depending on type and severity of anatomic abnormality some children with type I function normally, while type III has low survival rate Prevention no preventive measures are available at this time [medbullets.com]

Prognosis Long-term prognosis for persons with Arnold-Chiari I malformations is excellent. Full recovery from surgery may take several months. [encyclopedia.com]

Although studies of etiology are few, an increasing number of specific genetic syndromes are found to be associated with Chiari malformations. Management primarily targets supportive care and neurosurgical intervention when necessary. [medlink.com]

As almost all neonatal patients with Chari II have a myelomeningocele, it has been suggested that the underlying etiology is that of in utero CSF leak due to open spinal dysraphism. [radiopaedia.org]

[…] compression of cerebellum or disruption of cerebrospinal fluid flow through the foramen magnum The pathophysiology of Chiari type II malformation is more complex; although compressive mechanisms likely play a role, additional mechanisms may be functional Etiology [pathologyoutlines.com]

Contraindications Surgical decompression of foramen magnum is contraindicated when tonsillar herniation is caused by etiologies other than Chiari malformation, such as mass lesions in the posterior fossa or CSF hypotension syndrome. [emedicine.medscape.com]

He so established an etiological duality with a hydrodynamic cause for ACHSI and a mechanical cause due to traction for ACHSII, as it is currently for most authors. [raredr.com]

Epidemiology It used to be said that Chiari malformations are rare, but with routine use of MR imaging Chiari malformation is discovered with increasing frequency. [physio-pedia.com]

Erdener Özer, Dokuz Eylul University School of Medicine (Turkey) Terminology Chiari or Chiari’s malformation Epidemiology There are no population based studies on the incidence or prevalence of Chiari malformations From clinical series, prevalence has [pathologyoutlines.com]

Epidemiology Chiari type I Retrospective investigation of brain MRIs has found a prevalence of 1 in 1,280 individuals [ 5 ]. It tends to present earlier if there is syringomyelia. [patient.info]

Epidemiology of syringomyelia in Japan–the nationwide survey. Rinsho Shinkeigaku 1995; 35 : 1395–1397 (in Japanese). 15 Isu T. Surgical treatment for syryngomyelia associated with a Chiari malformation. [nature.com]

Epidemiology The most common Chiari malformation is type I and has been estimated to occur in 1 in 1000 births. The majority of these cases are asymptomatic. [emedicine.medscape.com]

Type II The pathophysiology of Chiari II is more complex. Although compressive mechanisms likely play a role, as in Chiari I, additional mechanisms may be operative in Chiari II. [physio-pedia.com]

Renewed effort to address current deficits in Chiari research involves work groups targeted at pathophysiology, symptoms and diagnosis, engineering and imaging analysis, treatment, pediatric issues, and related conditions. [medlink.com]

Symptoms of Chiari type I malformation develop due to compression of medulla and upper spinal cord, compression of cerebellum or disruption of cerebrospinal fluid flow through the foramen magnum The pathophysiology of Chiari type II malformation is more [pathologyoutlines.com]

[…] of 3 pathophysiological consequences of the disordered anatomy: (1) compression of medulla and upper spinal cord, (2) compression of cerebellum, and (3) disruption of CSF flow through foramen magnum. [emedicine.medscape.com]

Pathophysiology of primary spinal syringomyelia. Clinical article. J Neurosurg Spine. 2012 Nov;17(5):367-80. [ PubMed ] [Free full text] doi: 10.3171/2012.8.SPINE111059. Pratioparnawatr P, Timkao S, Tanapaisal C, Kanpittaya J, Jitpimolmard S. [apicareonline.com]

and Comlications Prognosis range widely depending on type and severity of anatomic abnormality some children with type I function normally, while type III has low survival rate Prevention no preventive measures are available at this time prevent neural [medbullets.com]

Figure 1 Ghaly Obstetric Guide to Arnold-Chiari malformation type 1 (GOGAC-1) In order to prevent the occurrence of a preventable catastrophe, the obstetric specialists need to raise awareness of the ACM-1 condition in pregnant patients to their primary [surgicalneurologyint.com]

PRS and anxiety symptoms should be explored in ACM patients to allow better prevention of psychosis and anxiety disorders. [ncbi.nlm.nih.gov]

It is most important to prevent neural complications during operations for idiopathic scoliosis with Chiari malformation and syringomyelia. [ci.nii.ac.jp]

Prevention There is no known way to prevent Chiari malformation. Genetic counseling may help parents of a child with this condition to determine the risk in future children. References: Chiari malformation fact sheet. [cancercarewny.com]