Presentation
The patients' records were retrospectively reviewed for demographic data, chief complaint and presenting symptoms, neurological and radiographic findings, treatment, outcome, and complications. [ncbi.nlm.nih.gov]
The clinical presentation depends on the site of the cavity and the involved structures [6] which ranges from asymptomatic presentation to cranial nerve palsy [7], vocal cord paralysis [8] and facial myokymia [9]. [beat-journal.com]
Entire Body System
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Pain
[…] heat and pain. [quintpub.com]
The case occurred in a 3.5-year-old girl who presented with a history of frequent bouts of severe pain in the lower neck and the shoulder blades. Simple decompression of the syrinx by open myelotomy resulted in prompt relief and reversal of symptoms. [ncbi.nlm.nih.gov]
It makes perfect sense for those who suffer with relentless pain. [pinterest.se]
Because of the loss of pain and temperature sensation in the hands and inner forearms, painless ulcer may follow a trauma. [nmihi.com]
The patient must be taught to avoid damage which may result from absence of pain. No drugs can retard or reverse the condition. Analgesics may be required for pain. Surgery Surgery is usually recommended where there are symptoms. [patient.info]
Respiratoric
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Stridor
A 10-year, 11-month-old boy who had had chronic stridor since birth died suddenly following the development of slowly progressive symptoms of respiratory obstruction due to vocal cord paralysis. [ncbi.nlm.nih.gov]
Children may rarely present with stridor even. [1] Paraesthasia of the face Due to the involvement of trigeminal nerve there could be bilateral or unilateral sensory impairment for pain and temperature on face. [1][6] Dysphagia Due to the palatal and [explainmedicine.com]
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Respiratory Abnormalities
Respiratory abnormalities failed to correlate with syrinx size. CONCLUSIONS: Severe abnormalities in respiratory rhythm generation during sleep occur in patients with syringobulbia. [ncbi.nlm.nih.gov]
Respiratory abnormalities failed to correlate with syrinx size. Conclusions: Severe abnormalities in respiratory rhythm generation during sleep occur in patients with syringobulbia. [neurology.org]
Gastrointestinal
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Hiccup
Hiccups caused by a neoplasm in the spinal cord are rare. [ncbi.nlm.nih.gov]
[…] that last up to 48 h, persistent hiccups that last for over 48 h and intractable hiccups are defined as hiccups that last more than 1 month. 2 Persistent and intractable singultus may cause severe discomfort, decreased physical strength and depression [casereports.bmj.com]
[…] complication following spinal cord injury - case report. ( 29485364 ) Mousele C....Constantoyannis C. 2018 4 Syringohydromyelia in association with syringobulbia and syringocephaly: case report. ( 26030334 ) Menezes A.H....Abode-Iyamah K. 2015 5 Intractable hiccups [malacards.org]
Psychiatrical
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Impulsivity
Syringobulbia clefts due to a dissection of the CSF under pressure from the fourth ventricle should be differentiated from the ascending syringobulbia which may occur from upward impulsive fluid movements in a syringomyelia. [ncbi.nlm.nih.gov]
Neurologic
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Nystagmus
Oscillopsia, nystagmus, and Horner syndrome are frequent manifestations of syringobulbia. Oscillopsia may be a disturbing symptom for the patient, whereas Horner syndrome is usually an asymptomatic finding. [ncbi.nlm.nih.gov]
[…] malformation with syringomyelia But very rare… 3-5% of patients with Chiari malformation 4 Symptoms Head and neck pain Snoring Diplopia Sensory disturbances Unsteady gait Dysphagia Paresthesias and numbness 5 Clinical Findings Cranial nerve deficits Nystagmus [slideplayer.com]
The Disease Database lists the following medical conditions that Syringobulbia may cause: * Bulbar palsy * Cerebellar syndrome * Dizzyness * Nystagmus Other Possible Causes of these Symptoms * Rapid involuntary eye movements * Speaking difficulty * Vertigo [checkorphan.org]
In syringobulbia, the individual frequently complains of dizziness and a sensation of spinning ( vertigo ), jerky eye movements ( nystagmus ), tongue wasting, uni- or bilateral facial sensory impairment, and speech impairment (dysphagia). [nmihi.com]
This is evidenced by dysphagia, pharyngeal and palatal weakness, asymmetric weakness and atrophy of the tongue, sensory loss primarily affecting pain and temperature sense in the distribution of the trigeminal nerve, and nystagmus. [quintpub.com]
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Myokymia
A patient with a 2-year history of progressive ataxia and tingling in the right hand had prominent facial myokymia. Magnetic resonance imaging revealed syringomyelia and syringobulbia. [ncbi.nlm.nih.gov]
The clinical presentation depends on the site of the cavity and the involved structures [6] which ranges from asymptomatic presentation to cranial nerve palsy [7], vocal cord paralysis [8] and facial myokymia [9]. [beat-journal.com]
[…] report. ( 8346478 ) Kudo H....Tamaki N. 1993 34 Syringobulbia: a surgical appraisal. ( 1479391 ) Morgan D....Williams B. 1992 35 Risk of sudden death during sleep in syringomyelia and syringobulbia. ( 1640236 ) NoguAcs M.A....Encabo H. 1992 36 Facial myokymia [malacards.org]
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Cranial Nerve Involvement
nerve involvement: With syringobulbia Most often unilateral XII: Tongue weakness & hemiatrophy IX - X: Dysphagia; Dysarthria XI: Weakness & wasting of sternomastoid & trapezius VII: Facial paresis Descending tract of V Reduced pain & temperature on lateral [neuromuscular.wustl.edu]
Interestingly, despite the syrinxes in their brainstems, neither patient had symptoms or neurological signs, such as breathing issues or cranial nerve involvement, directly attributable to those syrinxes. [conquerchiari.org]
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Oscillopsia
Oscillopsia, nystagmus, and Horner syndrome are frequent manifestations of syringobulbia. Oscillopsia may be a disturbing symptom for the patient, whereas Horner syndrome is usually an asymptomatic finding. [ncbi.nlm.nih.gov]
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
Posterior fossa decompression is a safe and effective treatment. [ncbi.nlm.nih.gov]
Prognosis
Treatment and prognosis When symptomatic, neurosurgical intervention may be required 1. Promoted articles (advertising) [radiopaedia.org]
Prognosis - Syringobulbia Not supplied. Treatment - Syringobulbia Not supplied. Resources - Syringobulbia Not supplied. [checkorphan.org]
The prognosis depends on the course of the underlying lesion. [books.google.ro]
The prognosis depends on the course of the underlying lesion. [books.google.com]
Livanainen, Prognosis of syringomyelia. Acta Neurol. Scand. 43 (1967), 61–68. Google Scholar Conway, L. W., Hydrodynamic studies in syringomyelia. J. Neurosurg. 27 (1967), 501–514. PubMed Google Scholar Cramer, F. [link.springer.com]
Etiology
Abstract The treatment of syringobulbia requires a precise diagnosis of the etiology. This allows a surgical procedure directed at treating the underlying etiology or decompressing the hindbrain. In most cases, symptoms can be greatly improved. [ncbi.nlm.nih.gov]
Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10coded.com]
Although post-traumatic syringomyelia has been wieldy described previously, traumatic brain injury has not been reported as the mechanism and etiology of isolated syringobulbia. [beat-journal.com]
Syringomyelia Treatment is Based on Etiology Asymptomatic syringomyelia patients with small syrinx cavities and no obvious etiology are best managed with watchful waiting and serial imaging exams. [csfinfo.org]
Etiology Etiology of syringomyelia often is associated with craniovertebral junction abnormalities. [emedicine.com]
Epidemiology
Epidemiology References: [1] [2] Epidemiological data refers to the US, unless otherwise specified. Etiology Syringomyelia is an abnormal fluid-filled dilation of the central canal of the spinal cord occurring as a result of impaired CSF flow. [amboss.com]
Associations Scoliosis Spina bifida Klippel-Feil syndrome Epidemiology There is a paucity of data on the prevalence of the condition in the West. [patient.info]
D. (2002) Taxonomy and epidemiology of spinal cord injury pain. In: Yezierski, R.P. and Burchiel, K. (eds.) [shiga-med.ac.jp]
General Epidemiology: Varied frequency 1.9 per 100,000 in Japan 8.4 per 100,000 in Western countries Pathology Cavitation: Fluid filled Surrounding tissue: Gliosis Location Spinal cord Usually cervical or thoracic: C2 to T9 Medulla Course: Chronic Children [neuromuscular.wustl.edu]
[…] meningeal inflammation Arachnoid scarring related to surgical trauma Subarachnoid space stenosis due to spinal neoplasm or vascular malformation Subarachnoid space stenosis, with possible scarring, related to disk and osteophytic disease Idiopathic Epidemiology [emedicine.com]
Pathophysiology
The pathophysiology is discussed. [ncbi.nlm.nih.gov]
Includes separate chapters focusing on individual neurologic disorders with detailed descriptions from pathophysiology to in-depth analysis. [books.google.ro]
NoguAcs M.A....Encabo H. 1992 36 Facial myokymia in syringobulbia. ( 2181981 ) Riaz G....Ghatak N. 1990 37 Syringomyelia and syringobulbia following tuberculous meningitis. ( 2192017 ) Schon F....Bowler J.V. 1990 38 Syringomyelia and syringobulbia: pathophysiology [malacards.org]
Pathophysiology Although many mechanisms for syrinx formation have been postulated, the exact pathogenesis is still unknown. Frequently cited theories are those of Gardner, William, and Oldfield. [emedicine.com]
Prevention
MRI detection of syringomyelia has led to earlier treatment of syringomyelia and prevention of upward extension of the cavity. This probably explains why syringobulbia is less frequently encountered at present. [ncbi.nlm.nih.gov]
Next, an allogeneic expansion duraplasty is performed to prevent recurrent adhesions in this area. [neupsykey.com]
[…] following medical conditions that Syringobulbia may cause: * Bulbar palsy * Cerebellar syndrome * Dizzyness * Nystagmus Other Possible Causes of these Symptoms * Rapid involuntary eye movements * Speaking difficulty * Vertigo * Vocal cord paralysis Prevention [checkorphan.org]
Builds a knowledge base of required skills for the necessary close observation and monitoring of the neuroscience patient to prevent complications and achieve the best outcomes. [books.google.ro]