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Query: UMLS:C0011168 (dysphagia)
 15,644 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Syringobulbia is a term which has been clinically applied to brain stem symptoms or signs in patients with syringomyelia. Syringobulbia clefts are found on investigation or at necropsy caused by cutting outwards of the CSF under pressure from the fourth ventricle into the medulla. These should be differentiated from the ascending syringobulbia which may occur from upward impulsive fluid movements in a previously established syringomyelia. Clinical analysis of 54 patients suggests that bulbar features are most often found with neither of the above mechanisms but are due to the effects of pressure differences acting downward upon the hind-brain with consequent distortion of the cerebellum and brainstem, traction on cranial nerves or indentation of the brain-stem by vascular loops. The commonest symptoms in the 54 patients were headache (35), vertigo (27), dysphonia or dysarthria (21), trigeminal paraesthesiae (27), dysphagia (24), diplopia (16), tinnitus (11), palatal palsy (11) and hypoglossal involvement (11). Careful attention to hydrocephalus is advisable before craniovertebral surgery, but the decompression of the hindbrain and the correction of craniospinal pressure dissociation remains the mainstay of surgical treatment. The results of careful surgery are good, 45 of the 54 cases reported improvement. Most of the reported deterioration occurred in a few patients who did conspicuously badly.
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PMID:Syringobulbia: a surgical appraisal. 147 91

Clinical, respiratory, and polysomnographic findings in three patients with syringomyelia and syringobulbia who developed severe respiratory complications are described. Neurological examination showed evidence of IXth and Xth cranial nerve involvement with dysphagia and dysphonia, but there were no complaints of serious sleep difficulties. Two patients died during sleep and the other was resuscitated during a nap. All patients showed moderate restrictive ventilatory defects with reduced maximal buccal pressures and one also showed a low ventilatory response to CO2 rebreathing. Protracted central, obstructive, and mixed apnoeas and hypopnoeas were commonly observed during sleep. There were no changes in heart rate during these events. A combination of respiratory and cardiovascular mechanisms might have been responsible for the severe complications described.
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PMID:Risk of sudden death during sleep in syringomyelia and syringobulbia. 164 Feb 36

The patient, a 78-year-old female with history of headache and progressive gait disturbance for almost one year, was admitted to our department because of dysphagia and dysphonia since three months before. Neurological examination revealed nystagmus, cerebellar ataxia, deafness, and vesical incontinence. No cerebral injuries were detected by computed tomography (CT) scan, although Paget's. Disease of Bone (PDB) was suggested, confirmed by biochemical and scintigraphic studies. The plain skull X-ray showed platybasia. As all the disarrangements were not explained by PDB complications alone, nuclear magnetic resonance imaging (MRI) was performed which demonstrated an Arnold-Chiari malformation (ACM) type I, with mild tonsillar herniation and anterior compression of the brainstem due to basilar impression, without syringomyelia. The association of PDB and ACM is a peculiarity seldom reported. The surgical approach was rejected, but the severity of symptoms and osteitis deformans biochemical activity needed a treatment; it was orientated to modify bone turnover using etidronate, a bisphosphonate, which induced clinical improvement and a decrease in serum alkaline phosphatase as well as in other bone resorption markers, without side effects. The good status and biochemical remission have been maintained a year later.
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PMID:[Paget's disease of bone and basilar impression associated with an Arnold-Chiari type-1 malformation]. 942 43

Two cases of acute presentation of cervical syringomyelia associated with Chiari malformation are reported. In the first case, dysphagia was the main symptom; the second patient had symptoms suggesting acute brain stem ischemia. The mechanism of this acute onset of the illness is discussed.
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PMID:[Acute onset syringomyelia: two cases]. 1145 90

We report a patient with syringobulbia extending to the pons, who could not open his mouth widely. He had been involved in the traffic accident at 16 years of age. Since them he had suffered numbness in the left neck and arm. At age 30, he became unable to open the mouth widely with pain in the left jaw joint. He also noted dysphagia and tinnitus. Neurologically, there were vocal cord paresis, dysesthesia of the face, ageusia and cerebellar ataxia all on the left side. Brain MRI revealed syringobulbia which extended to the pons. Spinal MRI revealed syringomyelia through the entire spinal cord. The syrinx of the spinal cord seemed to connect with the brainstem lesion. EMG of the masticatory muscles revealed paradoxical activity in the left masticatory muscles. We concluded that disturbance of jaw-opening in this case was caused by syringobulbia, the lesion of which could involve masticatory central pattern generator in the brainstem.
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PMID:[Disturbance of jaw-opening due to extension of syringobulbia to the pons--a case report]. 1260 83

The Chiari type 1 malformation is common. Unlike the Chiari type 2 and 3 malformations, it may remain latent for a long time, becoming symptomatic only in adulthood. The introduction of MRI has resulted in an increased number of diagnoses of this malformation in pediatric patients. It appears to be related to underdevelopment of the posterior cranial fossa. It must be differentiated from acquired tonsillar herniation, particularly when herniation results from intracranial hypotension; these cases are sometimes reported as acquired Chiari I malformation with spontaneous resolution. Tonsillar ectopia may cause symptoms by its direct effect on any or all of the medulla and the cerebellar and upper spinal cord. The most suggestive of the oculomotor disturbances is oscillopsia with downbeat nystagmus. Dysphonia and dysphagia are common. Potentially serious autonomic disturbances are also frequent: sleep apnea, respiratory failure, syncope and even sudden death. Another risk is syrinx formation, resulting from obstruction of CSF circulation in the cisterna magna. Syringomyelia is detected in 32 to 74% of patients with Chiari I malformation. Treatment is surgical. Posterior fossa decompression is achieved by suboccipital craniectomy combined with laminectomy of the upper cervical segments. Surgical intervention is indicated when the malformation is symptomatic and there is no doubt that it is the cause of the symptoms. When a Chiari I malformation is identified fortuitously on MRI, long-term monitoring is essential. The risk of developing symptoms increases over time. Patients should be advised not to participate in contact sports.
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PMID:[Chiari type 1 malformation and magnetic resonance imaging]. 1632 7

A 'syringomyelia-like' syndrome has been infrequently reported in neurological disorders such as Tangiers disease and lepromatous leprosy. This study reports a novel 'syringomyelia-like' syndrome in four adult male patients, which we have termed facial onset sensory and motor neuronopathy, or FOSMN syndrome, that appears to have a neurodegenerative aetiology. Clinical, neurophysiological and pathological data of four patients were reviewed, including the autopsy in one patient. Four male patients (mean age at onset 43), initially developed paraesthesiae and numbness in a trigeminal nerve distribution, which slowly progressed to involve the scalp, neck, upper trunk and upper limbs in sequential order. Motor manifestations, including cramps, fasciculations, dysphagia, dysarthria, muscle weakness and atrophy developed later in the course of the illness. Neurophysiological findings revealed a generalized sensory motor neuronopathy of caudally decreasing severity in all four patients. Autopsy in one patient disclosed loss of motoneurons in the hypoglossal nucleus and cervical anterior horns, along with loss of sensory neurons in the main trigeminal sensory nucleus and dorsal root ganglia. FOSMN syndrome appears to be a slowly progressive neurodegenerative disorder, whose pathogenesis remains to be determined.
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PMID:Facial onset sensory and motor neuronopathy (FOSMN syndrome): a novel syndrome in neurology. 1701 96

We present our experience in the diagnosis, surgical management and long-term follow-up of congenital and acquired osseous-ligamentous abnormalities or pathologies of the craniovertebral junction. The purpose of this study was: (i) to determine the incidence and degree of cervicomedullary compression in pediatric and young adult patients with congenital and acquired abnormalities, and (ii) to correlate cervicomedullary compression with other imaging and clinical factors to determine to what extend cervicomedullary compression is successfully treated with a posterior decompressive procedure, transoral decompression, and medical management. Between January 1995 and December 2004, 26 cases were managed in our department. These patients had: rheumatoid arthritis (RA) (3); traumatic injury (2); congenital basilar impression (5, in 2 cases a posteriorly oriented or retroflexed odontoid); infection (10); craniovertebral junction Pott's disease (9); os odonteideum (3); condylus tertius (1); and tumor (2). Six of the patients (23.1%) had syringomyelia. Only three (11.3%) were in the pediatric age group. Symptoms and signs included headache (72%), ataxia (38%), lower cranial nerve dysfunction (54%), quadriparesis (44%), hyperreflexia (76%), Hoffman positivity (72%), achilles clonus (72%) nystagmus (33%) and dysphagia (22%). The mean follow-up time was 44 months (range 3-85). Twelve (46.2%) had undergone posterior fossa decompression; seven (26.6%) had ventral decompression. Seven of the patients (26.6%) had medical management. The major morbidity included pharyngeal wound sepsis leading to dehiscence (3.8%), valopharyngeal insufficiency (3.8%), cerebrospinal fluid leakage (3.8%), postoperative macroglossia (3.8%) and inadequate anterior decompression (3.8%). Transient neurological deterioration occurred in two patients (7.6%). Our management paradigm will result in some neurologic improvements and limit the progression of symptoms. Patients with these pathologies are likely to show a good neurologic outcome when treatment, whether with or without surgery, is administered early in the course of the disease.
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PMID:Management of cervicomedullary compression in patients with congenital and acquired osseous-ligamentous pathologies. 1733 28

The case of a term, male neonate (birthweight 3785g) with cephalic presentation, Caesarean-section (C-section) delivery, and failure to thrive is reported. The infant presented with generalized hypotonia and respiratory failure immediately following birth. An initial diagnosis of hypoxic-ischemic encephalopathy was made. However, ventilator dependency and slow recovery of generalized tonus over the following weeks could not be explained. Late cervical magnetic resonance imaging showed extensive syringomyelia from C2 to C7. To the authors' knowledge, this is the first report of syringomyelia after a C-section delivery following cephalic presentation without any associated abnormalities. Follow-up at 2 years of age revealed no improvement on neurological examination: poor head control, difficulty swallowing, flaccid paralysis of upper limbs, and spasticity of lower limbs with exacerbated deep reflexes and spontaneous clonus. Difficulties in establishing the diagnosis and managing the case are discussed.
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PMID:Syringomyelia and chronic respiratory failure in a term infant delivered by Caesarean section. 1759 28

A 36-year-old woman presented with a history of change in voice of 1 month's duration and difficulty swallowing and episodes of nasal regurgitation of 6 days' duration. Examination revealed paralysis of the right vocal fold and the right side of the soft palate. On further investigation, she was found to have syringomyelia with syringobulbia and a Chiari type I malformation. She underwent foramen magnum decompression surgery and was followed up for 1 year. Her dysphagia and nasal regurgitation resolved, but no functional improvement was seen in either her vocal fold or palate.
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PMID:Syringomyelia with syringobulbia presenting as vocal fold paralysis. 1962 19


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