Presentation
Emphasis is placed on the clinical presentation and the possible differential diagnoses. Case Report A 45-year-old male presented with a history of headaches beginning at age 20 and lower left hemifacial pain for the past year. [quintpub.com]
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]
The case we presented is in some ways typical of PTS syndrome. This woman had an SCI 11 years before the finding of PTS. [nature.com]
Overview Snapshot A 32-year-old man presents with bilateral upper extremity weakness and sensory loss. Approximately 6 months ago, he was involved in a motor vehicle accident. [step1.medbullets.com]
Presentation Most present in the 20s and 30s but can present in childhood or later life. In the Japanese survey, 22% were asymptomatic, and half of cases were associated with Chiari malformation[1]. [patient.info]
Entire Body System
- Asymptomatic
In one study, all patients with greater than 12 mm of descent were symptomatic, whereas approximately 30% of those whose descent measured between 5 and 10 mm remained asymptomatic 1. [radiopaedia.org]
Serial magnetic resonance imaging studies over a 6-year period demonstrated spontaneous and complete resolution of the syrinx accompanied by an asymptomatic clinical course. The natural history of syringomyelia is highly unpredictable. [jstage.jst.go.jp]
Most commonly, the malformation remains asymptomatic throughout childhood, with symptoms becoming progressively more apparent as the patient ages. [consultant360.com]
The patient had been asymptomatic since then and had been seen by an outside neurologist frequently for the past 10 years. [surgicalneurologyint.com]
Asymptomatic patients usually do not require treatment. [nervous-system-diseases.com]
- Surgical Procedure
Keywords Arnold-Chiari malformation type I - foramen magnum - minimally invasive surgical procedures - neuroendoscopy - syringomyelia [thieme-connect.com]
That case differs from the present one in that a cervico-spinal decompression was performed, although the surgical procedure was not precisely described. [nature.com]
Indication for surgical intervention is controversial, as well as the surgical procedure to apply. [medicoebambino.com]
[…] the surgical procedure. [disability-benefits-help.org]
- Swelling
Hydrocephalus, when the circulation of CSF fluid is blocked and collects in the head, causing it to swell. Hydrocephalus can cause death if untreated. [medicalnewstoday.com]
It may happen when there is: Swelling of the covering of the spinal cord Meningitis Spinal cord injury Spinal tumor Tethered spinal cord Sometimes, it happens for reasons doctors don’t understand. They call this “idiopathic syringomyelia.” [webmd.com]
Frequent repositioning may be necessary because of your pain and swelling in lower extremities. [disability-benefits-help.org]
In general, a syrinx diameter of more than five millimeters (mm) and associated swelling (edema) generally indicate a worse prognosis. [my.clevelandclinic.org]
Loss of temperature and pain sensation in cervical dermatomes Claw hand Decreased muscle strength in hands, arms and shoulders Decreased to absent deep tendon reflexes in upper limb Muscle atrophy of hands, arms and shoulders Tenderness, erythema, swelling [wikidoc.org]
Respiratoric
- Hoarseness
Syringobulbia may cause vertigo, nystagmus, unilateral or bilateral loss of facial sensation, lingual atrophy and weakness, dysarthria, dysphagia, hoarseness, and sometimes peripheral sensory or motor deficits due to medullary compression. [msdmanuals.com]
He also gave history of hoarseness and cough while having food for the last one-month. A neurological examination revealed paralysis of 9th and 10th cranial nerves, dissociative sensory loss and diminished reflex in both upper limbs. [japi.org]
[…] include: headache (especially after sneezing, coughing, or straining) balance problems neck pain dizziness vision problems poor hand coordination numbness and tingling of the hands and feet change in bladder (pee) or bowel (poop) habits trouble swallowing hoarseness [kidshealth.org]
Weakness, numbness, balance problems and hoarseness can also occur. Possible eventual loss of bowel and bladder control can occur eventually in some cases. [neckandback.com]
The most common signs and symptoms are [5] Diplopia Episodic vertigo Trigeminal distribution pain Dysarthria Hoarseness in voice Unilateral tongue atrophy Analgesia and loss of temperature sensation on one side of the face Palatal and vocal cord paralysis [physio-pedia.com]
Gastrointestinal
- Dysphagia
The most important mechanism of recurrent aspiration pneumonia was dysphagia[ 14 ]. [mrmjournal.biomedcentral.com]
Syringobulbia and syringomyelia should be added to the list of diseases included in the article, as dysphagia, nausea, vomiting, and feeding difficulties may be early symptoms of these diseases. [n.neurology.org]
Syringobulbia may cause vertigo, nystagmus, unilateral or bilateral loss of facial sensation, lingual atrophy and weakness, dysarthria, dysphagia, hoarseness, and sometimes peripheral sensory or motor deficits due to medullary compression. [msdmanuals.com]
Signs and Symptoms Symptoms of Chiari II malformation/Arnold-Chiari malformation include dysfunction of CN 9 and 10 leading to dysphagia and respiratory distress. May present as a neurosurgical emergency in up to 20%. [csfinfo.org]
Compression of the hindbrain can cause weakness, paraesthesia, ataxia, cranial nerve palsies, dysphagia, dysphasia, palpitations, syncope, apnoea and sudden death. [patient.info]
- Failure to Thrive
In babies, the most common symptoms are a weak or absent cry, breathing difficulties, including stridor, arching of the neck, failure to thrive and feeding or swallowing difficulties. [physio-pedia.com]
[…] to thrive, or problems gaining or maintaining weight The occurrence of Chiari was once estimated to be about 1:1000 births, but with better imaging and diagnostic techniques physicians are finding it is actually much more common than was once thought [migraine.com]
[…] to thrive and opisthotonis This malformation is characterized by downward displacement of the medulla, fourth ventricle and cerebellum into the cervical spinal canal, as well as elongation of the pons and fourth ventricle. [aans.org]
- Hiccup
However, hiccups represent a rare clinical manifestation of this disorder. [scholars.houstonmethodist.org]
GOV'T INTRODUCTION: Hiccups caused by a neoplasm in the spinal cord are rare. [read.qxmd.com]
Musculoskeletal
- 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]
Disruption of CSF flow through foramen magnum probably accounts for the most common symptom, pain. Accordingly, headache and neck pain in Chiari I are often exacerbated by cough and Valsalva maneuver. Hydrocephalus occurs less frequently. [emedicine.medscape.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]
Clinical manifestations of types I-III include TORTICOLLIS ; opisthotonus; HEADACHE ; VERTIGO ; VOCAL CORD PARALYSIS ; APNEA ; NYSTAGMUS, CONGENITAL ; swallowing difficulties; and ATAXIA. [mesh.kib.ki.se]
Neurologic
- Headache
There have been reports of retro-orbital pain mimicking a cluster headache attack or paroxysmal hemicrania in the cervical cord, and dorsal medullary infarction presenting with retro-orbital pain.5,6 Our patient suffered cluster headache–like attacks [quintpub.com]
"Is Chiari type I malformation a reason for chronic daily headache?". Current Pain and Headache Reports. 11 (1): 53–5. doi : 10.1007/s11916-007-0022-x. PMID 17214922. ^ "Department of Neurological Surgery – University of Washington". [en.wikipedia.org]
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]
Supportive care such as headache and pain management, physical therapy or a reduction in activities can help manage symptoms. An operation may be recommended. This is referred to as a posterior fossa decompression. [asap.org]
- 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]
Clinical manifestations of types I-III include TORTICOLLIS; opisthotonus; HEADACHE; VERTIGO; VOCAL CORD PARALYSIS; APNEA; NYSTAGMUS, CONGENITAL; swallowing difficulties; and ATAXIA. [hon.ch]
Clinical manifestations of types I-III include TORTICOLLIS ; opisthotonus; HEADACHE ; VERTIGO ; VOCAL CORD PARALYSIS ; APNEA ; NYSTAGMUS, CONGENITAL ; swallowing difficulties; and ATAXIA. [mesh.kib.ki.se]
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]
- Vertigo
Clinical manifestations of types I-III include TORTICOLLIS; opisthotonus; HEADACHE; VERTIGO; VOCAL CORD PARALYSIS; APNEA; NYSTAGMUS, CONGENITAL; swallowing difficulties; and ATAXIA. [hon.ch]
Clinical manifestations of types I-III include TORTICOLLIS ; opisthotonus; HEADACHE ; VERTIGO ; VOCAL CORD PARALYSIS ; APNEA ; NYSTAGMUS, CONGENITAL ; swallowing difficulties; and ATAXIA. [mesh.kib.ki.se]
Syringobulbia may cause vertigo, nystagmus, unilateral or bilateral loss of facial sensation, lingual atrophy and weakness, dysarthria, dysphagia, hoarseness, and sometimes peripheral sensory or motor deficits due to medullary compression. [msdmanuals.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]
"Pathophysiology of syringomyelia associated with Chiari I malformation of the cerebellar tonsils. Implications for diagnosis and treatment". J Neurosurg. 80 (1): 3–15. doi:10.3171/jns.1994.80.1.0003. [wikidoc.org]
- Giddiness
She had presented with numbness and weakness of left upper limb for two years and episodes of bilateral frontal headache, giddiness and vertigo for one year. [apicareonline.com]
Workup
If no focal arachnopathy can be demonstrated after intensive neuroradiological workup (MRI with FLAIR, T1/T2 ± gadolinium-weighted images, cardiac-gated CINE-MRI for revealing CSF pulsation with additional 3D-CISS sequences) [22] but the syringomyelia [neupsykey.com]
Treatment
The type of treatment depends on what is causing the symptoms. [neuroaxis.com.au]
The carbamazepine and ergotamine treatment was continued and supplemented with amitriptyline (75 mg/day). [quintpub.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]
When a syrinx is identified without symptoms, it should be monitored but no treatment is needed. When symptoms of syringomyelia or syringobulbia occur, treatment can be performed to relieve pressure on the spinal cord to prevent further damage. [docshealth.com]
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]
Prognosis
Prevention, Complications Prognosis dependent on etiology and severity Complications neuropathic pain bulbar symptoms in patients with syringobulbia [step1.medbullets.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]
Outlook / Prognosis What is the prognosis (outlook) for syringomyelia? It’s important to remember that syringomyelia has a wide range of symptoms, severity and causes. [my.clevelandclinic.org]
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]
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]
Etiology
[…] polyradiculoneuropathy (AIDP) Treatment Surgical posttraumatic decompression if indicated surgically correct underlying condition e.g., posterior fossa decompression in chiari I malformation Prognosis, Prevention, Complications Prognosis dependent on etiology [step1.medbullets.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]
Etiology They may be congenital (90%) or acquired in etiology. [radiopaedia.org]
The etiology and pathogenesis of the disease is largely unknown, although syrinx cavities have now been described in up to 22% of posttraumatic injuries and have been associated with Arnold-Chiari malformation. [journal.chestnet.org]
Etiology Arnold-chiari malformation, especially type I (most common cause) [1] Posttraumatic SCI (3–4% of patients with SCI develop symptomatic syringomyelia) Postinflammatory: transverse myelitis, arachnoiditis Postinfectious (meningitis) Intramedullar [amboss.com]
Epidemiology
[…] can compress ventral horn motor neurons bilateral flaccid paralysis may involve descending hypothalamic fibers to preganglionic sympathetic neurons of T1-T4 Horner's syndrome Associated conditions Arnold-Chiari malformation spinal trauma malignancy Epidemiology [step1.medbullets.com]
Epidemiology Epidemiological data refers to the US, unless otherwise specified. [amboss.com]
In this article Aetiology Associations Epidemiology Presentation Investigations Management Complications Prognosis Syringomyelia and Syringobulbia In this article Aetiology Associations Epidemiology Presentation Investigations Management Complications [patient.info]
Pathophysiology
The pathophysiology underpinning the enlargement of syringes, however, may have a common theme. Across the years, imaging studies have helped elucidate the pathophysiology underpinning syrinx enlargement. [ajnr.org]
Hypothesis on the pathophysiology of syringomyelia based on simulation of cerebrospinal fluid dynamics. J Neurol Neurosurg Psychiatry 2003;74(3):344-7. PMID 12588922 11 Cleland J. [medlink.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]
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]
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 prevent neural [medbullets.com]
Diabetic neuropathy Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) Treatment Surgical posttraumatic decompression if indicated surgically correct underlying condition e.g., posterior fossa decompression in chiari I malformation Prognosis, Prevention [step1.medbullets.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]