A motor neuron disease marked by progressive weakness of the muscles innervated by cranial nerves of the lower brain stem. Clinical manifestations include dysarthria, dysphagia, facial weakness, tongue weakness, and fasciculations of the tongue and facial muscles. The adult form of the disease is marked initially by bulbar weakness which progresses to involve motor neurons throughout the neuroaxis. Eventually this condition may become indistinguishable from AMYOTROPHIC LATERAL SCLEROSIS. Fazio-Londe syndrome is an inherited form of this illness which occurs in children and young adults. (Adams et al., Principles of Neurology, 6th ed, p1091; Brain 1992 Dec;115(Pt 6):1889-1900)
A degenerative disorder affecting upper MOTOR NEURONS in the brain and lower motor neurons in the brain stem and SPINAL CORD. Disease onset is usually after the age of 50 and the process is usually fatal within 3 to 6 years. Clinical manifestations include progressive weakness, atrophy, FASCICULATION, hyperreflexia, DYSARTHRIA, dysphagia, and eventual paralysis of respiratory function. Pathologic features include the replacement of motor neurons with fibrous ASTROCYTES and atrophy of anterior SPINAL NERVE ROOTS and corticospinal tracts. (From Adams et al., Principles of Neurology, 6th ed, pp1089-94)
A medical specialty concerned with the use of physical agents, mechanical apparatus, and manipulation in rehabilitating physically diseased or injured patients.
Diseases characterized by a selective degeneration of the motor neurons of the spinal cord, brainstem, or motor cortex. Clinical subtypes are distinguished by the major site of degeneration. In AMYOTROPHIC LATERAL SCLEROSIS there is involvement of upper, lower, and brainstem motor neurons. In progressive muscular atrophy and related syndromes (see MUSCULAR ATROPHY, SPINAL) the motor neurons in the spinal cord are primarily affected. With progressive bulbar palsy (BULBAR PALSY, PROGRESSIVE), the initial degeneration occurs in the brainstem. In primary lateral sclerosis, the cortical neurons are affected in isolation. (Adams et al., Principles of Neurology, 6th ed, p1089)
Skilled treatment that helps individuals achieve independence in all facets of their lives. It assists in the development of skills needed for independent living.
Neurons which activate MUSCLE CELLS.
Restoration of human functions to the maximum degree possible in a person or persons suffering from disease or injury.
An oxidoreductase that catalyzes the reaction between superoxide anions and hydrogen to yield molecular oxygen and hydrogen peroxide. The enzyme protects the cell against dangerous levels of superoxide. EC 1.15.1.1.

Antiganglioside antibody in patients with Guillain-Barre syndrome who show bulbar palsy as an initial symptom. (1/48)

OBJECTIVES: To identify valuable antiganglioside antibodies that support the diagnosis of Guillain-Barre syndrome (GBS) and its variants in patients showing bulbar palsy as an initial symptom. METHODS: Medical records of 602 patients with GBS or its variants were reviewed. Fifteen patients had bulbar palsy as an initial symptom. Serum antibodies against GM1, GM1b, GD1a, GalNAc-GD1a, GT1a, and GQ1b were examined in 13 of them. RESULTS: Serum antiganglioside antibodies were positive in 11 (85%) patients. IgG anti-GT1a (n=8; 62%) and anti-GM1b (n=7; 54%) antibodies were often present, whereas all the patients had low or no anti-GM1 antibody activity. High anti-GD1a and anti-GQ1b IgG antibody titres were also present in some patients, but most had higher IgG antibody titres to GM1b or GT1a. All five patients with high IgG antibody titre to GM1b or GT1a only had had antecedent diarrhoea. Some patients with pharyngeal-cervical-brachial weakness (PCB) had IgG antibody to GT1a which did not cross react with GQ1b. Other patients with PCB had antibody to GT1a which cross reacted with GQ1b or antibody to GM1b, but anti-GM1b and anti-GT1a antibodies were not associated with the presence of bulbar palsy. All the patients who had no IgG antiganglioside antibodies recovered completely. CONCLUSIONS: Measurement of serum IgG anti-GT1a and anti-GM1b antibodies gives helpful support for the diagnosis of GBS and its variants when there is early involvement of the oropharyngeal function independently of other neurological findings which appear as the illness progresses.  (+info)

Worster-Drought syndrome, a mild tetraplegic perisylvian cerebral palsy. Review of 47 cases. (2/48)

A retrospective case-note analysis was undertaken of 47 children with a congenital upper motor neurone bulbar palsy (excluding pure speech dyspraxia) to clarify the phenotype of Worster-Drought syndrome (WDS) and to record its associated features and complications. The results revealed that the study children had significant bulbar problems (with 80% still needing a modified diet and a similar number using augmentative communication methods at last review). There were also high rates of predictable bulbar complications (86% had dribbling, 60% had glue ear, gastro-oesophageal reflux in 40%, history of poor nutrition in 40% and aspiration in 40%). Most of the children had additional complex impairments (91% had mild pyramidal tetraplegia, 81% learning difficulties, 60% congenital defects, 41% neuropsychiatric problems and 28% epilepsy). Over half of the children had significant medical problems in the first year, but mean age at diagnosis was 6 years. There were no obvious causes in pregnancy or birth. Six children had a family history of WDS and 32% (12/37) had abnormal neuroimaging including five with bilateral perisylvian polymicrogyria. In our experience, WDS is not uncommon, is relatively easily diagnosed and is crucial not to miss as the management of these children's multiple impairments is complex and requires a careful team approach. WDS falls clearly within the cerebral palsies as a syndrome that includes motor impairment arising from static damage to the brain in early life. The common presence of cognitive, behavioural and seizure impairments strongly supports the cerebral cortical (presumably perisylvian) localization. Its core elements are a suprabulbar paresis, a mild spastic tetraplegia and a significant excess of cognitive and behavioural impairments and epilepsy. The complete overlap in phenotype between WDS and the bilateral perisylvian syndrome leads us to propose that they are the same condition. WDS is startlingly absent from epidemiological studies of the cerebral palsies and rarely diagnosed, presumably because of lack of clinical awareness of the condition and lack of major gross motor impairments.  (+info)

Motor neuron diseases in the university hospital of Fortaleza (Northeastern Brazil): a clinico-demographic analysis of 87 cases. (3/48)

In this retrospective (1980-1998) study, we have analyzed clinico-demographically, from the records of the University Hospital of Fortaleza (Brazil), a group of 87 patients showing signs and symptoms of motor neuron diseases (MNDs). Their diagnosis was determined clinically and laboratorially. The WFN criteria were used for amyotrophic lateral sclerosis (ALS) diagnosis. The clinico-demographic analysis of the 87 cases of MNDs showed that 4 were diagnosed as spinal muscular atrophy (SMA), 5 cases as ALS subsets: 2 as progressive bulbar paralysis (PBP), 2 as progressive muscular atrophy (PMA) and 1 as monomelic amyotrophy (MA), and 78 cases of ALS. The latter comprised 51 males and 27 females, with a mean age of 42.02 years. They were sub-divided into 4 groups according to age: from 15 to 29 years (n= 17), 30 to 39 years (n= 18), 40 to 69 years (n= 39) and 70 to 78 years (n= 4). From the 78 ALS patients, 76 were of the classic sporadic form whilst only 2 were of the familial form. The analysis of the 87 patients with MNDs from the University Hospital of Fortaleza showed a predominance of ALS patients, with a high number of cases of juvenile and early onset adult sporadic ALS.  (+info)

Non-invasive screening for surgical intracranial lesions. (4/48)

The value and reliability of the combined results of skull radiographs, electroencephalography, echoencephalography, isotope angiography, and brain scanning in 147 patients suspected of having an intracranial space occupying lesions are analysed. The overall accuracy of the technique was 79%. No false negatives were found. The advantages of adopting the system proposed by the authors in everyday clinical work is discussed.  (+info)

Rehabilitation for postpolio sequelae. (5/48)

BACKGROUND: Postpolio sequelae (PPS) are new, late manifestations that occur many years after the initial poliomyelitis infection. Recurrence of symptoms and fear of reactivation of the polio virus is particularly distressing to polio survivors. OBJECTIVE: This article outlines the diagnosis, pathophysiology, and management of PPS disabilities using a case vignette. DISCUSSION: Clinical features of PPS include fatigue, joint and muscle pain, new muscular weakness and bulbar symptoms. Diagnosis can be complicated particularly in nonparalytic cases of poliomyelitis. Disabilities in PPS may not be obvious to the observer but significantly affect the quality of life of the PPS patient. Previous rehabilitation intervention focussed on physical effort and determination to overcome disability at all costs. The treatment in PPS is now modified, and aggressive physical measures that may exacerbate muscle weakness are avoided. Most disabilities in PPS can be well managed with rehabilitation interventions that address limitations in patient activities of daily living, mobility and cardiopulmonary fitness.  (+info)

Subacute bulbar palsy as the initial sign of follicular thyroid cancer. (6/48)

We report a 64-year-old woman with follicular thyroid cancer found by subacute bulbar palsy. Progressive bulbar palsy (PBP) was considered the most likely diagnosis, because no abnormal finding was detected on brain CT and blood test except for the decrease of free T4. An echogram of the thyroid revealed a small nodule which was shown to be class IIb by fine needle biopsy. However, 201Tl scintillation examination showed skull base metastasis. Follicular thyroid cancer sometimes seems to manifest as a distant metastasis with no clinically evident thyroid lesion. This case suggested the importance of a detailed survey for malignancy, when subacute bulbar palsy is seen.  (+info)

Congenital oculo-bulbar palsy. (7/48)

A girl developed progressive weakness of bulbar and ocular muscles starting before the age of two years. Electromyography revealed a widespread subclinical myopathy. An intercostal muscle biopsy showed complex abnormalities including occasional neurofilamentous accumulations and honeycomb-like membranous material in terminal axons. Endplates were small and some secondary synaptic clefts were abnormally deep. Acetylcholine receptors extended unusually deeply into the clefts of the junctional folds. Muscle fibres showed subsarcolemmal vacuolation at some places. This form of congenital oculo-bulbar palsy does not appear to have been described previously.  (+info)

The Scottish Motor Neuron Disease Register: a prospective study of adult onset motor neuron disease in Scotland. Methodology, demography and clinical features of incident cases in 1989. (8/48)

The Scottish Motor Neuron Disease Register (SMNDR) is a prospective, collaborative, population based study of motor neuron disease (MND) in Scotland. The register started in January 1989 with the aim of studying the clinical and epidemiological features of MND by prospectively identifying incident patients. It is based on a system of registration by recruitment from multiple sources, followed by the collection of complete clinical data and follow up, mainly through general practitioners. In this report the register's methodology and the demography and incidence data for the first year of study are presented. One hundred and fourteen newly diagnosed patients were identified in 1989 giving a crude incidence for Scotland of 2.24/100,000/year. Standardised incidence ratios showed a non-significant trend towards lower rates in north eastern regions and island areas.  (+info)

Bulbar palsy, progressive refers to a condition where there is a gradual loss of muscle function in the face, tongue, and throat due to damage to the brainstem. This condition is also known as progressive bulbar palsy (PBP).

The brainstem is responsible for controlling many of the body's automatic functions, including breathing, heart rate, and swallowing. When the brainstem is damaged, it can lead to a range of symptoms, including weakness or paralysis of the muscles in the face, tongue, and throat.

The symptoms of progressive bulbar palsy may include:

* Difficulty speaking or slurred speech
* Weakness or paralysis of the facial muscles
* Difficulty swallowing (dysphagia)
* Weight loss due to difficulty eating and drinking
* Fatigue and weakness
* Decreased reflexes

Progressive bulbar palsy can be caused by a variety of conditions, including:

* Brainstem stroke or bleeding
* Brain tumors
* Multiple sclerosis
* Amyotrophic lateral sclerosis (ALS)
* Other neurodegenerative disorders

There is no cure for progressive bulbar palsy, but treatment may include:

* Speech therapy to improve communication skills
* Swallowing therapy to reduce the risk of choking or pneumonia
* Physical therapy to maintain muscle strength and function
* Medications to manage symptoms such as pain, weakness, or fatigue

The prognosis for progressive bulbar palsy is generally poor, with many individuals experiencing significant decline in their quality of life and eventually succumbing to the disease. However, the rate of progression can vary greatly depending on the underlying cause of the condition.

ALS is caused by a breakdown of the nerve cells responsible for controlling voluntary muscle movement, leading to muscle atrophy and loss of motor function. The disease can affect anyone, regardless of age or gender, but it is most common in people between the ages of 55 and 75.

The symptoms of ALS can vary from person to person, but they typically include:

* Muscle weakness or twitching
* Muscle wasting or atrophy
* Loss of motor function, such as difficulty walking, speaking, or swallowing
* Slurred speech or difficulty with language processing
* Weakness or paralysis of the limbs
* Difficulty with balance and coordination
* Fatigue and weakness
* Cognitive changes, such as memory loss and decision-making difficulties

There is currently no cure for ALS, but there are several treatments available to help manage the symptoms and slow the progression of the disease. These include:

* Riluzole, a medication that reduces the amount of glutamate in the brain, which can slow down the progression of ALS
* Physical therapy, to maintain muscle strength and function as long as possible
* Occupational therapy, to help with daily activities and assistive devices
* Speech therapy, to improve communication and swallowing difficulties
* Respiratory therapy, to manage breathing problems
* Nutritional support, to ensure adequate nutrition and hydration

The progression of ALS can vary greatly from person to person, but on average, people with the disease live for 2-5 years after diagnosis. However, some people may live for up to 10 years or more with the disease. The disease is usually diagnosed through a combination of medical history, physical examination, and diagnostic tests such as electromyography (EMG) and magnetic resonance imaging (MRI).

There is ongoing research into the causes of ALS and potential treatments for the disease. Some promising areas of research include:

* Gene therapy, to repair or replace the faulty genes that cause ALS
* Stem cell therapy, to promote the growth of healthy cells in the body
* Electrical stimulation, to improve muscle function and strength
* New medications, such as antioxidants and anti-inflammatory drugs, to slow down the progression of ALS

Overall, while there is currently no cure for ALS, there are several treatments available to help manage the symptoms and slow the progression of the disease. Ongoing research offers hope for new and more effective treatments in the future.

MND is often fatal, usually within 2-5 years of diagnosis. There is currently no cure for MND, although various treatments and therapies can help manage the symptoms and slow its progression.

The most common types of MND are amyotrophic lateral sclerosis (ALS) and primary lateral sclerosis (PLS). ALS is characterized by rapid degeneration of motor neurons in the brain and spinal cord, leading to muscle weakness and paralysis. PLS is a slower-progressing form of MND that affects only the lower motor neurons.

MND can be caused by a variety of factors, including genetics, age, and exposure to toxins. It is often diagnosed through a combination of medical history, physical examination, and diagnostic tests such as electromyography (EMG) and magnetic resonance imaging (MRI).

There is ongoing research into the causes and potential treatments for MND, including stem cell therapy, gene therapy, and drugs that target specific molecules involved in the disease process.

The term infantile progressive bulbar palsy is used to describe progressive bulbar palsy in children. The ICD-11 lists ... Progressive bulbar palsy symptoms can include progressive difficulty with talking and swallowing. Patients can also exhibit ... Progressive bulbar palsy (PBP) is a medical condition. It belongs to a group of disorders known as motor neuron diseases. PBP ... The patient developed progressive bulbar palsy, became dependent on a respirator, and had two episodes of cardiac arrest. The ...
... is a rare type of progressive bulbar palsy that occurs in children. The disease exists in ... Infantile progressive bulbar palsy. Piña-Garza, J. Eric (2013). Fenichel's Clinical Pediatric Neurology E-Book: A Signs and ...
Progressive bulbar palsy Rea, Paul (2015). "5 - Hindbrain (Rhombencephalon)". Essential Clinical Anatomy of the Nervous System ... In contrast, pseudobulbar palsy is a clinical syndrome similar to bulbar palsy but in which the damage is located in upper ... "Bulbar Palsy". Palsy. Archived from the original on 2011-04-27. (All articles with dead external links, Articles with dead ... Bulbar palsy refers to a range of different signs and symptoms linked to impairment of function of the glossopharyngeal nerve ( ...
Families with multiple cases of BVVL and, more generally, multiple cases of infantile progressive bulbar palsy can show ... "Infantile progressive bulbar palsy with deafness". Brain & Development. 24 (7): 732-735. doi:10.1016/S0387-7604(02)00085-2. ... "Bulbar Palsy, Progressive, with Sensorineural Deafness" (PDF). The Catalogue for Transmission Genetics in Arabs Database, ... March 2010). "Brown-Vialetto-Van Laere syndrome, a ponto-bulbar palsy with deafness, is caused by mutations in c20orf54". ...
Progressive bulbar palsy (PBP) is a disease that attacks the nerves supplying the bulbar muscles. Infantile progressive bulbar ... palsy is progressive bulbar palsy in children. Both lampreys and hagfish possess a fully developed medulla oblongata. Since ... In modern clinical usage, the word bulbar (as in bulbar palsy) is retained for terms that relate to the medulla oblongata, ... The word bulbar can refer to the nerves and tracts connected to the medulla, and also by association to those muscles ...
SBMA was first described in Japanese literature in 1897 by Hiroshi Kawahara in a case report detailing progressive bulbar palsy ... Update: July 3, 2014 Kennedy, W. R.; Alter, M.; Sung, J. H. (1968). "Progressive proximal spinal and bulbar muscular atrophy of ... Further signs and symptoms include: Neurological Bulbar signs: bulbar muscles are those supplied by the motor nerves from the ... "Clinical Features of Spinal and Bulbar Muscular Atrophy". "OMIM Entry - # 313200 - SPINAL AND BULBAR MUSCULAR ATROPHY, X-LINKED ...
... progressive bulbar palsy. John R. Bentson, 83, American neuroradiologist, complications from COVID-19. Moshe Brawer, 101, ... Mrigendra Nath Maiti, 77, Indian politician, West Bengal MLA (since 2011). Sean Malone, 50, American progressive metal bassist ...
... progressive bulbar palsy, pseudobulbar palsy, and monomelic amyotrophy (MMA). ALS itself can be classified in a few different ... progressive bulbar palsy, and progressive muscular atrophy were all different forms of the same disease, neurone being a ... progressive bulbar palsy, progressive muscular atrophy, and primary lateral sclerosis. In the United Kingdom and Australia, the ... Bulbar onset is associated with a worse prognosis than limb-onset ALS; a population-based study found that bulbar-onset ALS has ...
... just as one can get accustomed to progressive bulbar palsy. But to start it afresh - that leads to trouble." Wikimedia Commons ...
... a lot of attention since he had been away from the spotlight for a while after losing his voice due to Progressive bulbar palsy ...
Benjamin Brooks of Carolinas Medical Center said Basnight had progressive bulbar palsy and that, while someone could die from ...
... a stimulant Progressive bulbar palsy, a motor neuron disease Picture by picture, two TV pictures side-by-side, similar to ...
They include amyotrophic lateral sclerosis (ALS), progressive bulbar palsy (PBP), pseudobulbar palsy, progressive muscular ... "Progressive Muscular Atrophy - an overview , ScienceDirect Topics". www.sciencedirect.com. Retrieved 24 June 2022. "Motor ... Bulbar symptoms, including difficulty speaking (dysarthria), difficulty swallowing (dysphagia), and excessive saliva production ... Motor neuron disease describes a collection of clinical disorders, characterized by progressive muscle weakness and the ...
Progressive bulbar palsy, a form of motor neuron disease, is associated with combined lesions of the hypoglossal nucleus and ... Bulbar palsy Jugular foramen syndrome Dale Purves (2012). Neuroscience. Sinauer Associates. p. 726. ISBN 978-0-87893-695-3. M. ... Hui, Andrew C. F.; Tsui, Ivan W. C.; Chan, David P. N. (2009-06-01). "Hypoglossal nerve palsy". Hong Kong Medical Journal = ... Keane, James R. (1996-06-01). "Twelfth-Nerve Palsy: Analysis of 100 Cases". Archives of Neurology. 53 (6): 561-566. doi:10.1001 ...
... bulbar palsy, progressive MeSH C10.574.562.500 - muscular atrophy, spinal MeSH C10.574.562.500.750 - spinal muscular atrophies ... bulbar palsy, progressive MeSH C10.668.460.500 - muscular atrophy, spinal MeSH C10.668.460.500.750 - spinal muscular atrophies ... chronic progressive external MeSH C10.597.622.447.690 - supranuclear palsy, progressive MeSH C10.597.622.669 - paraplegia MeSH ... chronic progressive external MeSH C10.292.562.750.500 - supranuclear palsy, progressive MeSH C10.292.562.775 - ophthalmoplegia ...
... (FLD), also called progressive bulbar palsy of childhood, is a very rare inherited motor neuron disease of ... It causes progressive bulbar paralysis due to involvement of motor neurons of the cranial nerve nuclei. The most frequent ... The gene encodes the intestinal riboflavin transporter (hRFT2).[citation needed] Symptoms of Fazio-Londe include bulbar palsy, ... "Progressive bulbar paralysis of childhood. A reappraisal of Fazio-Londe disease". Brain: A Journal of Neurology. 115 (Pt 6): ...
This includes amyotrophic lateral sclerosis (ALS), progressive bulbar palsy (PBP), pseudobulbar palsy, progressive muscular ...
Progressive bulbar palsy). He was the first clinician to practise muscle biopsy, with an invention he called "l'emporte-pièce ... Duchenne muscular dystrophy Erb-Duchenne palsy Duchenne-Aran disease Essai sur la brûlure (1833) De l'Électrisation localisée ...
Bell's palsy, partial facial paralysis Bulbar palsy, impairment of cranial nerves Cerebral palsy, a neural disorder caused by ... also known as wasting palsy Progressive supranuclear palsy, a degenerative disease Squatter's palsy, a common name for ... I. Macpherson JM, Gordon AJ Squatter's palsy British Medical Journal, 1983 Kumaki DJ. The facts of Kathmandu: squatter's palsy ... a disorder affecting the ability to move the eyes Erb's palsy, also known as brachial palsy, involving paralysis of an arm ...
These include progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS). PSP predominantly involves rigidity, early ... falls, bulbar symptoms, and vertical gaze restriction; it can also be associated with frontotemporal dementia symptoms. CBS ... Parkinson-plus syndromes, such as progressive supranuclear palsy and multiple system atrophy, must also be considered and ruled ... progressive supranuclear palsy, and dementia with lewy bodies. Differential diagnosis can be narrowed down with careful history ...
... "slowly progressive ptosis and extraocular palsy, and weakness of the masseter, facial, and bulbar muscles, as well as distal ... People with this condition exhibit symptoms susch as progressive muscle weakness affecting the eyes, face, and pharynx which ... Oculopharyngodistal myopathy is a rare genetic disorder characterized by progressive muscle weakness affecting various parts of ...
Motor neuron disease Tidy C (21 October 2021). Knott L (ed.). "Bulbar and Pseudobulbar Palsy. What is Bulbar Palsy?". Patient. ... PMID 7484639.] Graham KC, Spiegel DR (2008). "Pseudobulbar palsy and affect in a case of progressive multifocal ... Corticobulbar tract Bulbar palsy, a similar syndrome caused by the damage of lower motor neurons. ... artery of percheron infarct Progressive supranuclear palsy Amyotrophic lateral sclerosis Parkinson's disease and related ...
Sleep apnea Sleep disorders Impaired coordination Severe cases may develop all the symptoms and signs of a bulbar palsy ... Syringomyelia is most often chronic progressive degenerative disorder characterized by a fluid-filled cyst located in the ...
VII nerve palsies and bulbar palsy). Some cases presented with flaccid paralysis alone. In northern Australia, all melioidosis ... Results of a chest X-ray can range from diffuse nodular infiltrates in those with septic shock to progressive consolidation ... Clinical signs include: unilateral upper motor neuron limb weakness, cerebellar signs, and cranial nerve palsies (VI, ...
Prion diseases Progressive hemifacial atrophy Progressive multifocal leukoencephalopathy Progressive supranuclear palsy ... syndrome Sleep apnea Sleeping sickness Slurred speech Snatiation Sotos syndrome Spasticity Spina bifida Spinal and bulbar ... see Progressive supranuclear palsy Stiff-person syndrome Stroke Sturge-Weber syndrome Stuttering Subacute sclerosing ... body disease Cytomegalovirus Infection Dandy-Walker syndrome Dawson disease De Morsier's syndrome Dejerine-Klumpke palsy ...
... bulbar MeSH C02.182.600.700.800 - postpoliomyelitis syndrome MeSH C02.256.076.045 - adenovirus infections, human MeSH C02.256. ... bell palsy MeSH C02.256.466.175 - chickenpox MeSH C02.256.466.245 - cytomegalovirus infections MeSH C02.256.466.245.150 - ... progressive interstitial, of sheep MeSH C02.782.815.616.850 - simian acquired immunodeficiency syndrome MeSH C02.782.815.616. ... bulbar MeSH C02.782.687.359.764.650 - postpoliomyelitis syndrome MeSH C02.782.687.359.855 - swine vesicular disease MeSH ...
This neuropathy begins with paralysis and numbness of the soft palate and pharynx as well as bulbar weakness several days to ... It has also been associated with Bell's palsy, and vestibular neuritis. HSV-2 frequently lies within lumbosacral ganglia and is ... resulting in a progressive sensory loss associated with painful paresthesias of the upper limbs, or a mixed sensorimotor ... During acute infection, both direct peripheral nervous involvement, most commonly bilateral facial palsy, and an acute ...
X linked Sixth nerve palsy Sjögren-Larsson syndrome Sjögren's syndrome Skandaitis Skeletal dysplasia brachydactyly Skeletal ... brachymorphia syndrome Sphingolipidosis Spielmeyer-Vogt disease Spina bifida Spina bifida hypospadias Spinal and bulbar ... lower extremity predominance 2 Spinal muscular atrophy with pontocerebellar hypoplasia Spinal muscular atrophy with progressive ... deficiency Summitt syndrome Superior mesenteric artery syndrome Super mesozoic-dysentery complex Supranuclear ocular palsy ...
Clinicians frequently think of the symptoms of botulism in terms of a classic triad: bulbar palsy and descending paralysis, ... The muscle paralysis is progressive; it usually begins at the hindquarters and gradually moves to the front limbs, neck, and ...
Cerebral palsy is a term referring to a non-progressive developmental neuromotor disorder with an upper motor neuron lesion ... In the bulbar form, speech is affected before the limbs; here handwriting and typing on keyboard-style devices are frequently ... Depending on the location of the brain lesion, individuals with cerebral palsy can have a wide variety of gross and fine motor ... "Prevalence of Cerebral Palsy". cerebralpalsy.org. Archived from the original on 12 July 2017. Retrieved 26 April 2018. " ...
Furthermore, progressive supranuclear palsy and parkinsonism are associated with only minor olfactory problems. These findings ... How the bulbar neural circuit transforms odor inputs to the bulb to the bulbar responses that are sent to the olfactory cortex ... "Olfactory testing differentiates between progressive supranuclear palsy and idiopathic Parkinson's disease". Neurology. 43 (5 ...
SUOX Supranuclear palsy, progressive atypical; 260540; MAPT Supranuclear palsy, progressive; 601104; MAPT Supravalvar aortic ... SLC4A1 Spinal and bulbar muscular atrophy of Kennedy; 313200; AR Spinal muscular atrophy, distal, autosomal recessive, 4; ... progressive myoclonic 1; 254800; CSTB Epilepsy, progressive myoclonic 1B; 612437; PRICKLE1 Epilepsy, progressive myoclonic 2B; ... GBA Gaze palsy, horizontal, with progressive scoliosis; 607313; ROBO3 Geleophysic dysplasia; 231050; ADAMTSL2 Generalized ...
The term pseudobulbar (pseudo- + bulbar) came from the idea that the symptoms seemed similar to those caused by a bulbar lesion ... PBA is generally associated with later stages of the disease (chronic progressive phase). PBA in MS patients is associated with ... Levitt S (2013). Treatment of Cerebral Palsy and Motor Delay. Wiley. ISBN 9781118699782. Archived from the original on 20 ... cerebral palsy, autism, epilepsy, and migraines. This may lead to avoidance of social interactions for the patient, which in ...
DIAGNOSES Progressive bulbar palsy. INTERVENTIONS:. Chinese herbal medicine and acupuncture. OUTCOMES:. After 4 months of ... RATIONALE Progressive bulbar palsy (PBP) is a type of motor neuron disease (MND). The main symptoms include dysarthria, ... Herbal medicine and acupuncture relieved progressive bulbar palsy for more than 3 years: A case report. ... Herbal medicine and acupuncture relieved progressive bulbar palsy for more than 3 years: A ...
... neuron diseases are a group of neurologic disorders that present in adult life and are characterized primarily by progressive ... Progressive bulbar palsy - Pure bulbar involvement. * Progressive muscular atrophy - Pure lower motor neuron degeneration ... Newall AR, Orser R, Hunt M. The control of oral secretions in bulbar ALS/MND. J Neurol Sci. 1996 Aug. 139 Suppl:43-4. [QxMD ... Severe bulbar involvement may also preclude oral feeding due to significant aspiration and, therefore, a PEG feeding tube may ...
Other MNDs include progressive muscular atrophy, progressive bulbar palsy, and primary lateral sclerosis. ... Most persons who are first diagnosed with progressive muscular atrophy, progressive bulbar palsy, or primary lateral sclerosis ...
The key features are progressive ponto-bulbar palsy and bilateral sensorineural deafness. A complex neurological phenotype with ... The key features are progressive ponto-bulbar palsy and bilateral sensorineural deafness. A complex neurological phenotype with ... Brown-Vialetto-Van Laere syndrome, a ponto-bulbar palsy with deafness, is caused by mutations in c20orf54 Peter Green 1 , ... Ponto-bulbar palsy with deafness (Vialetto-Van Laere syndrome). Puri V, Rohtagi A, Parihar PS. Puri V, et al. Indian Pediatr. ...
Pontobulbar palsy with deafness. *Progressive bulbar palsy with sensorineural deafness. *Riboflavin transporter deficiency ... Brown-Vialetto-Van Laere syndrome, a ponto-bulbar palsy with deafness, is caused by mutations in c20orf54. Am J Hum Genet. 2010 ... Damage to these nerves causes paralysis of the muscles controlled by them, a condition called pontobulbar palsy. Nerves in the ... As a result of pontobulbar palsy, people with riboflavin transporter deficiency neuronopathy can have breathing problems; ...
... is characterized by late-childhood-onset slowly progressive cerebellar ataxia and distal sensorimotor axonal neuropathy. Gaze ... Progressive bulbar palsy; childhood-onset sensorineural deafness; childhood-onset neuronopathy that is more prominent as upper ... Slowly progressive ataxia; depressed tendon reflexes; dysarthria; muscle weakness; lower-limb spasticity; optic nerve atrophy; ... Slowly progressive disorder beginning in late childhood to early adulthood (ages 13-27 years) ...
I have progressive bulbar palsy. After right upper back right molar crown dental work dec2011 noticed speech slurring. By June ...
... are a group of progressive neurological disorders that destroy motor neurons, the cells that control skeletal muscle activity ... Progressive bulbar palsy (PBP), also known as progressive bulbar atrophy, attacks the lower motor neurons connected to the ... progressive bulbar palsy, primary lateral sclerosis, progressive muscular atrophy, spinal muscular atrophy, Kennedys disease, ... Some symptoms of stroke and myasthenia gravis are similar to those of progressive bulbar palsy and must be ruled out prior to ...
A 49-year-old man with progressive bulbar palsy and respiratory failure].. Motoi Y; Satoh K; Matsumine H; Wakiya M; Mori H; ... 7. [A 70-year-old man with a progressive gait disturbance and gaze palsy].. Nakamura T; Yoritaka A; Sumino S; Suzuki H; Mori H ... and marked pseudo-bulbar palsy].. Noda K; Kobayashi T; Matsuoka S; Takanashi M; Kanazawa A; Mizuno Y. No To Shinkei; 2005 Jan; ... 3. [A 65-year-old man with Parkinsonism, gaze palsy, and dementia].. Mizuno Y; Yokochi F; Ohta S; Mori H; Takubo H. No To ...
Bulbar Palsy Bulbar Palsy, Progressive, Of Childhood Childhood Progressive Bulbar Palsy Fazio-Londe Disease Fazio-Londe ... Childhood Progressive Bulbar Palsy Narrower Concept UI. M0337043. Terms. Childhood Progressive Bulbar Palsy Preferred Term Term ... Bulbar Palsy Broader Concept UI. M000618614. Terms. Bulbar Palsy Preferred Term Term UI T030274. Date03/30/1974. LexicalTag NON ... Bulbar Palsy, Progressive Preferred Concept UI. M0015879. Scope Note. A motor neuron disease marked by progressive weakness of ...
Progressive bulbar palsy G1229 Other motor neuron disease G128 Other spinal muscular atrophies and related syndromes G129 ... Spastic diplegic cerebral palsy G802 Spastic hemiplegic cerebral palsy G803 Athetoid cerebral palsy G8100 Flaccid hemiplegia ... Progressive multifocal leukoencephalopathy A8181 Kuru A8182 Gerstmann-Straussler-Scheinker syndrome A8183 Fatal familial ... Progressive supranuclear ophthalmoplegia [Steele-Richardson-Olszewski] G232 Striatonigral degeneration G238 Other specified ...
Subtypes of the disease are defined by location of damage, as in progressive bulbar palsy, or preferential involvement of upper ... The average duration of life after onset of symptoms of ALS is three years, encompassing a progressive course of increasing ... ALS is a progressive fatal neuromuscular disease, the prototype and most common disorder in the class of motor neuron diseases ... leading to muscle atrophy and progressive paralysis. ...
... and pupillary abnormality and bulbar palsy (34%) were present. Almost all the patients had a monophasic remitting course and ... reviewed detailed clinical profiles and laboratory findings for 62 cases of BBE diagnosed by the strict criteria of progressive ...
Progressive Bulbar Palsy (PBP). PBP can cause the patient to lose their ability to speak completely, making it difficult to ... Bulbar Onset ALS. As with other Different Types of Motor Neuron Disease, Bulbar Onset ALS is progressive, meaning that the ... Nutritional support is important for people with Bulbar Onset ALS in order to keep their muscles nourished.. Living with Bulbar ... Progressive Muscular Atrophy (PMA). PMA can affect different muscles in different parts of the body, including the arms, hands ...
... progressive bulbar palsy, corticobasal syndrome, Huntington disease, Alzheimer s disease, or other related adult-onset ... Aging-related progressive neurological disorders include frontotemporal dementia, Lou Gehrig s disease, and Alzheimer s disease ... To see if the drug [11C]ER176 can show inflammation in the brain in people with certain progressive neurological disorders ... Have other major neurological or medical diseases that may cause progressive weakness or cognitive dysfunction, such as ...
... progressive bulbar palsy, corticobasal syndrome, Huntington disease, Alzheimer s disease, or other related adult-onset ... Aging-related progressive neurological disorders include frontotemporal dementia, Lou Gehrig s disease, and Alzheimer s disease ... To see if the drug [11C]ER176 can show inflammation in the brain in people with certain progressive neurological disorders ... Have other major neurological or medical diseases that may cause progressive weakness or cognitive dysfunction, such as ...
Progressive bulbar palsy (PBP). People with amyotrophic lateral sclerosis (ALS) frequently also suffer from progressive bulbar ... 4. Progressive muscular atrophy. Atrophy of the skeletal muscles is a rare form of muscular dystrophy. Muscle wasting, most ... Bulbar weakness accompanied by dysarthria and dysphagia, limb weakness, muscular wasting and fasciculation, and, most ... palsy (PBP), a disorder that affects the brain stem. It results in persistent episodes of choking as well as problems ...
Bulbar Palsies, Progressive Palsies, Progressive Bulbar Palsy, Progressive Bulbar Progressive Bulbar Palsies Progressive Bulbar ... Palsy, Progressive Bulbar. Paralysis, Bulbar. Progressive Bulbar Palsies. Progressive Bulbar Palsy. Progressive Bulbar Palsy of ... Bulbar Palsies. Bulbar Palsies, Progressive. Bulbar Palsy. Bulbar Palsy, Progressive, Of Childhood. Bulbar Paralyses. Bulbar ... Bulbar Paralyses Bulbar Paralysis Palsies, Bulbar Palsy, Bulbar Paralysis, Bulbar Childhood Progressive Bulbar Palsy - Narrower ...
Bulbar Palsy Bulbar Palsy, Progressive, Of Childhood Childhood Progressive Bulbar Palsy Fazio-Londe Disease Fazio-Londe ... Childhood Progressive Bulbar Palsy Narrower Concept UI. M0337043. Terms. Childhood Progressive Bulbar Palsy Preferred Term Term ... Bulbar Palsy Broader Concept UI. M000618614. Terms. Bulbar Palsy Preferred Term Term UI T030274. Date03/30/1974. LexicalTag NON ... Bulbar Palsy, Progressive Preferred Concept UI. M0015879. Scope Note. A motor neuron disease marked by progressive weakness of ...
Bulbar Palsy, Progressive. Sweden 3. Clinical syndrome of ALS (amyotrophic lateral sclerosis) ... 2. Amyotrophic lateral sclerosis with bulbar paralysis and degeneration in Golls columns: a contribution to the pathology of ... Amyotrophic lateral sclerosis with bulbar paralysis and degeneration in Golls columns: a contribution to the pathology of the ...
Bulbar weakness. No. No. Left VII cranial nerve palsy. No. Bilateral VII cranial nerve palsy; bulbar weakness; tetraparesis. ... Yes (progressive, asymmetric, bilateral). Yes (left progressive to bilateral, asymmetric). Yes (progressive, asymmetric, ... Progressive asymmetric 4- limb weakness. ICU care; mechanical ventilation; tracheostomy; feeding support. ...
Progressive Bulbar Palsy Medicine & Life Sciences 21% * Flavin Mononucleotide Medicine & Life Sciences 19% ... These patients commonly present with bulbar palsy, hearing loss, muscle weakness, and respiratory symptoms in infancy or later ... These patients commonly present with bulbar palsy, hearing loss, muscle weakness, and respiratory symptoms in infancy or later ... These patients commonly present with bulbar palsy, hearing loss, muscle weakness, and respiratory symptoms in infancy or later ...
Progressive muscular atrophy. *Progressive bulbar palsy *Fazio-Londe. *Infantile progressive bulbar palsy ... Chronic traumatic encephalopathy: a progressive degenerative disease associated with multiple concussions and other forms of ...
Bulbar Palsy, Progressive 1 0 Death, Sudden, Cardiac 1 0 Note: The number of publications displayed in this table will differ ...
2) PROGRESSIVE BULBAR PALSY:-. Involvement of motor nuclei in the lower brainstem. ... Bulbar onset- bulbar or pseudobulbar features-25% dysphagic or dysarthria.. *In both limb -onset and bulbar-onset disease the ... There is a primary involvement of the bulbar muscles and hence the jaw muscles, facial muscles, tongue larynx and pharynx are ... Only involvement of both the corticospinal and cortico bulbar. *It gives rise to UMN type of presentation in the extremities ...
Progressive Bulbar Palsy. *Prostate Cancer - Hormone Refractory Disease - or with visceral metastases ...
Bulbar Palsy, Progressive. Sweden 3. Clinical syndrome of ALS (amyotrophic lateral sclerosis) ... 2. Amyotrophic lateral sclerosis with bulbar paralysis and degeneration in Golls columns: a contribution to the pathology of ... Amyotrophic lateral sclerosis with bulbar paralysis and degeneration in Golls columns: a contribution to the pathology of the ...
Progressive bulbar palsy (PBP).. *Pseudobulbar palsy.. Differences and Similarities Between ALS and MS. Differences. Here are ... The disease is progressive, which means that it gets worse over time, affecting nerve cells in the nervous system (brain and ...
  • This group includes diseases such as amyotrophic lateral sclerosis, progressive bulbar palsy, primary lateral sclerosis, progressive muscular atrophy, spinal muscular atrophy, Kennedy's disease, and post-polio syndrome. (nih.gov)
  • People with amyotrophic lateral sclerosis (ALS) frequently also suffer from progressive bulbar palsy (PBP), a disorder that affects the brain stem. (miska.co.in)
  • Amyotrophic lateral sclerosis (ALS) is a progressive and generally fatal neurodegenerative disorder . (symptoma.com)
  • Amyotrophic lateral sclerosis (ALS) is a fatal neu- romuscular disease causing rapid degeneration of motor 23 Dec 2015 People with ALS now have more options to deal with a distressing set of symptoms called bulbar signs. (web.app)
  • however, efficacious diagnostic This patient presented with progressive bulbar dysfunction, manifesting as nasal Although the referral diagnosis was amyotrophic lateral sclerosis (ALS), bulbar palsy usually progresses to ALS. (web.app)
  • In any case of adult onset bulbar symptoms, amyotrophic lateral sclerosis (ALS) is a suggested diagnosis. (web.app)
  • DOI10.1111/1460- Selective neuronal populations are affected leading to symptoms which are prominently motor in amyotrophic lateral sclerosis (ALS) or Huntington's disease (ALS) affects the intrinsic tongue muscles differently when the symptoms begin ALS (limb onset) and 5 patients with progressive bulbar ALS (cranial onset). (web.app)
  • Hon har progressiv bulbär pares, så hennes form av ALS börjar i mun och Amyotrophic lateral sclerosis (ALS) associated with superoxide dismutase 1 including the bulbar (speech, chewing, swallowing) and respiratory muscles. (web.app)
  • the most common form of MND), primary lateral sclerosis, progressive muscular atrophy and progressive bulbar palsy. (racgp.org.au)
  • If you think you may have Progressive Muscular Atrophy, contact your doctor as soon as possible. (herbal-care-products.com)
  • Progressive muscular atrophy involves lower motor neurons only. (web.app)
  • Damage to these nerves causes paralysis of the muscles controlled by them, a condition called pontobulbar palsy. (medlineplus.gov)
  • In this disorder of unknown cause, motor neurons of the brain and spinal cord degenerate, leading to muscle atrophy and progressive paralysis. (nih.gov)
  • However, most experts believe that the toxin does not cause progressive paralysis when applied in tiny doses for cosmetic use and they claim there is no evidence of it causing long-term damage to motor neurons or any other neuromuscular structure. (wellnaturalhealth.com)
  • Case of bulbar paralysis of nine months' duration. (nih.gov)
  • Report of a case of bulbar paralysis. (nih.gov)
  • Hereditary neuropathy with liability to pressure palsies (HNPP) is characterized by recurrent acute sensory and motor neuropathy in a single or multiple nerves. (nih.gov)
  • bulbar hereditary motor sensory neuronopathy (Fazio-Londe's disease). (web.app)
  • Bulbar onset- bulbar or pseudobulbar features-25% dysphagic or dysarthria. (sarvanshikhalora.in)
  • Pseudobulbar palsy. (newlifeoutlook.com)
  • Amyotrofisk lateralskleros (ALS), även kallad Lou Gehrigs sjukdom eller klassisk Pseudobulbar pares, som delar många symptom på progressiv bulbar pares, Typer av motor neuron sjukdomar inklusive ALS och mer Eftersom lägre motor neuroner påverkas är progressiv svaghet ett vanligt symptom. (web.app)
  • ALS involves upper and lower motor neurons and presents as an idiopathic, progressive degeneration of anterior horn cells and their associated neurons, resulting in progressive muscle weakness, atrophy, and fasciculations. (medscape.com)
  • Bulbar weakness (or bulbar palsy) refers to bilateral impairment of function of the lower cranial nerves IX, X, XI and XII, which occurs due to lower motor neuron lesion either at nuclear or fascicular level in the medulla or from bilateral lesions of the lower cranial nerves outside the brain-stem. (nih.gov)
  • Bulbar weakness is often associated with difficulty in chewing, weakness of the facial muscles, dysarthria, palatal weakness and regurgitation of fluids, dysphagia, and dysphonia. (nih.gov)
  • Early symptoms of ALS usually include muscle weakness or stiffness in a limb or muscles of the mouth or throat (so-called bulbar muscles). (nih.gov)
  • A motor neuron disease marked by progressive weakness of the muscles innervated by cranial nerves of the lower brain stem. (nih.gov)
  • The adult form of the disease is marked initially by bulbar weakness which progresses to involve motor neurons throughout the neuroaxis. (nih.gov)
  • These patients commonly present with bulbar palsy, hearing loss, muscle weakness, and respiratory symptoms in infancy or later in childhood. (elsevier.com)
  • Given its progressive and debilitating nature and the lack of cure, management of MND is focused on symptomatic relief, optimising quality of life and timely palliative care involvement. (racgp.org.au)
  • Subtypes of the disease are defined by location of damage, as in progressive bulbar palsy, or preferential involvement of upper or lower motor neuron, as in primary lateral sclerosis or spinal muscular atrophy. (nih.gov)
  • There is a primary involvement of the bulbar muscles and hence the jaw muscles, facial muscles, tongue larynx and pharynx are affected. (sarvanshikhalora.in)
  • Bulbar involvement results in spastic dysarthria and dysphagia . (symptoma.com)
  • We do not know why some individuals develop bulbar involvement as the first symptom of ALS, while others develop bulbar involvement only as a late 11 May 2020 Approximately 30% of patients with ALS present with bulbar signs and symptoms (Table 2). (web.app)
  • Hepresented with slurred speech progressing to anarthria and bilateral palsy of lower motor cranial nerves within 24 hour. (bezmialem.edu.tr)
  • We experienced with the patient that CADASIL may present with unusual progressive manifestations and bilateral simultaneous infarctions. (bezmialem.edu.tr)
  • Nutritional support is important for people with Bulbar Onset ALS in order to keep their muscles nourished. (herbal-care-products.com)
  • Adult-onset motor neuron diseases are a group of neurologic disorders that present in adult life and are characterized primarily by progressive degeneration and loss of motor neurons. (medscape.com)
  • Fazio-Londe disease is a progressive bulbar palsy with onset in childhood that presents with hypotonia and respiratory insufficiency (summary by Bosch et al. (nih.gov)
  • The average duration of life after onset of symptoms of ALS is three years, encompassing a progressive course of increasing disability. (nih.gov)
  • As with other Different Types of Motor Neuron Disease, Bulbar Onset ALS is progressive, meaning that the symptoms get worse over time as more motor neurons are damaged and destroyed. (herbal-care-products.com)
  • Medications such as riluzole and edaravone can help slow down the progression of Bulbar Onset ALS. (herbal-care-products.com)
  • Living with Bulbar Onset ALS can be difficult for both the person affected and their family. (herbal-care-products.com)
  • Progressive bulbar palsy (PBP) , also known as progressive bulbar atrophy, attacks the lower motor neurons connected to the brain stem. (nih.gov)
  • RATIONALE Progressive bulbar palsy (PBP) is a type of motor neuron disease (MND). (bvsalud.org)
  • Motor neurone disease (MND) is the term for a group of progressive, debilitating, neurodegenerative disorders that affect various aspects of a patient's life, including speech, swallowing, breathing and limb function. (racgp.org.au)
  • ALS is a progressive fatal neuromuscular disease, the prototype and most common disorder in the class of motor neuron diseases. (nih.gov)
  • Aging-related progressive neurological disorders include frontotemporal dementia, Lou Gehrig s disease, and Alzheimer s disease. (nih.gov)
  • Motor neuron disease (MND) is a progressive condition that progressively damages parts of nervous system or degeneration of upper and lower motor neuron. (sarvanshikhalora.in)
  • The disease is progressive, which means that it gets worse over time, affecting nerve cells in the nervous system (brain and spinal cord), causing loss of muscle control. (newlifeoutlook.com)
  • Motor Neuron Disease Motorneuronsjukdom Svensk definition Sjukdomstillstånd ALS eller Motor Neuron Disease, MND, är gemensam en beteckning för en grupp Bulbar palsy refers to a range of different signs and symptoms linked to I denna artikel vill jag prata om vad som är ALS (sjukdom). (web.app)
  • Besides ophthalmoplegia and ataxia, disturbance of consciousness was frequent (74%), and facial diplegia (45%), Babinski's sign (40%) and pupillary abnormality and bulbar palsy (34%) were present. (nih.gov)
  • We report a 35 year-old migraine-free patient with unusual presentation with acute simultaneous multiple subcortical infarctions causing progressive bulbar palsy as the initial manifestation. (bezmialem.edu.tr)
  • progressive impairment of cognition, behavior, vision, hearing, and motor function follow the initial symptoms and often lead to total disability within six months to two years. (nih.gov)
  • all symptoms are progressive over decades. (nih.gov)
  • 10. [A study of bulbar, upper extremity, and lower extremity motor function preservation at the time that respiratory symptoms appear in ALS]. (nih.gov)
  • Progressiv Bulbar Pares Bulbar palsy refers to a range of different signs and symptoms linked to impairment of Vanliga symtom vid ALS - tal och sväljsvårigheter. (web.app)
  • Motor neuron diseases (MNDs) are a group of progressive neurological disorders that destroy motor neurons, the cells that control skeletal muscle activity such as walking, breathing, speaking, and swallowing. (nih.gov)
  • Herbal medicine and acupuncture relieved progressive bulbar palsy for more than 3 years: A case report. (bvsalud.org)
  • Réflexions sur la maladie décrite par M. Duchenne sous le nom de paralysie musculaire progressive des lèvres, de la langue et du voile du palais. (nih.gov)
  • ALS2 patients suffer from a slowly progressive form of ALS. (symptoma.com)
  • There have even been reports of patients developing bulbar palsy after repeated treatment. (wellnaturalhealth.com)
  • When the condition begins in children or young adults, sensorineural hearing loss usually occurs first, followed by signs of pontobulbar palsy. (medlineplus.gov)
  • To see if the drug [11C]ER176 can show inflammation in the brain in people with certain progressive neurological disorders compared to healthy adults. (nih.gov)
  • This group includes diseases such as amyotrophic lateral sclerosis, progressive bulbar palsy, primary lateral sclerosis, progressive muscular atrophy, spinal muscular atrophy, Kennedy's disease, and post-polio syndrome. (nih.gov)
  • Amyotrophic lateral sclerosis and other motor neuron diseases are characterized by steady, relentless, progressive degeneration of corticospinal tracts, anterior horn cells, bulbar motor nuclei, or a combination. (msdmanuals.com)
  • It is a fatal disorder and is characterized by progressive skeletal muscle weakness and wasting or atrophy (ie, amyotrophy), spasticity, and fasciculations as a result of degeneration of the UMNs and LMNs, culminating in respiratory paralysis. (medscape.com)
  • Damage to these nerves causes paralysis of the muscles controlled by them, a condition called pontobulbar palsy. (medlineplus.gov)
  • Riboflavin transporter deficiency is a progressive neurodegenerative disease characterized by paralysis of the cranial nerves, sensorineural deafness, and signs of damage to other nerves. (nih.gov)
  • In this disorder of unknown cause, motor neurons of the brain and spinal cord degenerate, leading to muscle atrophy and progressive paralysis. (nih.gov)
  • The key features are progressive ponto-bulbar palsy and bilateral sensorineural deafness. (nih.gov)
  • Subtypes of the disease are defined by location of damage, as in progressive bulbar palsy, or preferential involvement of upper or lower motor neuron, as in primary lateral sclerosis or spinal muscular atrophy. (nih.gov)
  • Spinocerebellar ataxia with axonal neuropathy type 1 (SCAN1) is characterized by late-childhood-onset slowly progressive cerebellar ataxia and distal sensorimotor axonal neuropathy. (nih.gov)
  • PLS is a rare, idiopathic neurodegenerative disorder that primarily involves the UMNs, resulting in progressive spinobulbar spasticity. (medscape.com)
  • In adults, because most of the cases presenting with these pure bulbar symptoms represent so-called bulbar-onset ALS and eventually develop widespread symptoms typically seen in ALS, some authors consider this disorder to be a subset of ALS. (medscape.com)
  • ALS is a progressive fatal neuromuscular disease, the prototype and most common disorder in the class of motor neuron diseases. (nih.gov)
  • Motor neuron diseases (MNDs) are a group of progressive neurological disorders that destroy motor neurons, the cells that control skeletal muscle activity such as walking, breathing, speaking, and swallowing. (nih.gov)
  • Adult-onset motor neuron diseases are a group of neurologic disorders that present in adult life and are characterized primarily by progressive degeneration and loss of motor neurons. (medscape.com)
  • 4. [A 49-year-old man with progressive bulbar palsy and respiratory failure]. (nih.gov)
  • Aging-related progressive neurological disorders include frontotemporal dementia, Lou Gehrig s disease, and Alzheimer s disease. (nih.gov)
  • To see if the drug [11C]ER176 can show inflammation in the brain in people with certain progressive neurological disorders compared to healthy adults. (nih.gov)
  • 20. [A 61-year-old man with progressive gait disturbance, freezing, and vertical gaze paresis who developed esophagus cancer]. (nih.gov)
  • 17. [A 65-year-old man with rigid-bradykinetic parkinsonism, vertical gaze palsy, difficulty of eye-lid opening, and marked pseudo-bulbar palsy]. (nih.gov)
  • Spinal Muscular Atrophies (SMAs) Spinal muscular atrophies include several types of hereditary disorders characterized by skeletal muscle wasting due to progressive degeneration of anterior horn cells in the spinal cord and. (msdmanuals.com)
  • HSP, also known as familial spastic paraplegias or Strumpell-Lorrain disease, comprises a clinically and genetically heterogeneous group of hereditary disorders characterized by slowly progressive spastic paraparesis. (medscape.com)
  • In bulbar palsies, only the cranial nerve motor nuclei in the brain stem (bulbar nuclei) are affected. (msdmanuals.com)
  • 2. [A 70-year-old man with a progressive gait disturbance and gaze palsy]. (nih.gov)
  • 19. [A 65-year-old man with Parkinsonism, gaze palsy, and dementia]. (nih.gov)
  • A 65-year-old man with a history of gastric cancer who presented progressive loss of vision, memory loss and consciousness disturbance]. (nih.gov)