Results 31 to 40 of about 7,103 (220)

Progressive Bulbar Palsy

Developmental Medicine & Child Neurology, 1969
SUMMARYProgressive bulbar palsy is very rare in childhood. A probable case diagnosed in Greece is presented. After a short review of the literature the clinical and laboratory findings are described. The main clinical features were progressive affection of the bulbar nuclei over one year without involvement of the long tracts.
Gregoriou, M., Matsaniotis, N., Papadakis, G.   +2 more
openaire   +3 more sources

Adrenomyeloneuropathy with bulbar palsy: A rare association

Annals of Indian Academy of Neurology, 2014
Adrenomyeloneuropathy (AMN) is a variant of adrenoleukodystrophy (ALD), an X-linked recessive peroxisomal disorder associated with accumulation of very long chain fatty acids (VLCFA). Mutations of this gene lead to abnormal peroxisomal β-oxidation, which
Vishal Annaji Chafale, Satish Arunkumar Lahoti, Atanu Biswas, Arijit Roy, Asit Kumar Senapati   +4 more
doaj   +1 more source

Progressive Bulbar Palsy Care: Exploring the Potential of Functional MRI for Early Detection

Yemeni Journal for Medical Sciences
Progressive bulbar palsy (PBP) is a neurodegenerative disorder impacting motor neurons involved in speech, swallowing, and breathing. Early diagnosis is challenging due to limited diagnostic tools. Functional MRI (fMRI) holds promise for detecting early biomarkers and tracking disease progression, potentially improving clinical management.
Rita Komalasari
openalex   +3 more sources

Broad clinical phenotypes associated with TAR-DNA binding protein (TARDBP) mutations in amyotrophic lateral sclerosis [PDF]

, 2010
The finding of TDP-43 as a major component of ubiquitinated protein inclusions in amyotrophic lateral sclerosis (ALS) has led to the identification of 30 mutations in the transactive response-DNA binding protein (TARDBP) gene, encoding TDP-43.
A Yokoseki, BA Hosler, C Lomen-Hoerth, C Vance, Christopher J. McDermott, D Neary, DR Rosen, E Buratti, E Buratti, E Kabashi, Emily F. Goodall, F Gros-Louis, GM Ringholz, H Daoud, Hannah C. Hollinger, I Gijselinck, IF Wang, IR Mackenzie, J Kirby, J Sreedharan, J. Robin Highley, Janine Kirby, JJ Kew, Judith A. Hartley, Karen E. Morrison, L Benajiba, L Corrado, M Morita, M Neumann, MJ Strong, MJ Winton, NJ Rutherford, P Kuhnlein, PA Mercado, Pamela J. Shaw, Paul G. Ince, PM Andersen, R Bo Del, R Lemmens, Rachel Hibberd, RJ Guerreiro, Rudo Masanzu, SH Ou, Stephen B. Wharton, VM Deerlin Van, William Smith, YM Ayala   +46 more
core   +1 more source

Acute Thyrotoxic Bulbar Myopathy with Encephalopathic Behaviour: An Uncommon Complication of Hyperthyroidism

Case Reports in Endocrinology, 2013
Objective. Acute thyrotoxic bulbar palsy is rare, severe, and rapidly progressive. We describe a case of thyrotoxicosis with bulbar palsy, encephalopathy, and pyramidal tract dysfunction. Case Report. 64-year-old white male with toxic multinodular goiter
Neeraja J. Boddu, Sridhar Badireddi, Karl David Straub, John Schwankhaus, Rajani Jagana   +4 more
doaj   +1 more source

Brown-Vialetto-Van Laere syndrome [PDF]

, 2008
The Brown-Vialetto-Van Laere syndrome (BVVL) is a rare neurological disorder characterized by progressive pontobulbar palsy associated with sensorineural deafness. Fifty-eight cases have been reported in just over 100 years.
Sivakumar Sathasivam
core   +1 more source

Disease duration of progression is helpful in identifying isolated bulbar palsy of amyotrophic lateral sclerosis [PDF]

BMC Neurology, 2021
Abstract Background Compared with typical bulbar onset amyotrophic lateral sclerosis (ALS), isolated bulbar palsy (IBP), an often under-understood variant of ALS, is characterized by symptoms confined to bulbar region for extended periods and relative preservation of limb and ventilation function.
Huagang Zhang, Lu Chen, Jinzhou Tian, Dongsheng Fan   +3 more
openaire   +3 more sources

Fazio Londe syndrome: A treatable disorder

Annals of Indian Academy of Neurology, 2015
Fazio Londe Syndrome is a rare neurological disorder presenting with progressive bulbar palsy with respiratory failure. Initially considered to have an unrelenting course, is now found to be due to mutations in the SLC52A3 gene which encodes the ...
Poovazhagi Varadarajan, Vimal Thayanathi, Leema C Pauline   +2 more
doaj   +1 more source

Correlation Between Maximal Tongue Pressure and Swallowing Function in Spinal and Bulbar Muscular Atrophy

Frontiers in Neurology, 2021
Background: Spinal and bulbar muscular atrophy (SBMA) is an X-lined motor neuron disease characterized by progressive muscle weakness, bulbar palsy, and dysphagia.
Dae-Won Gwak, Seung-Hwan Jung, Yu-Sun Min, Jin-Sung Park, Hee-Jin Cho, Donghwi Park, Min Woo Hong, Min-Gu Kang   +7 more
doaj   +1 more source

Apraxia and motor dysfunction in corticobasal syndrome [PDF]

, 2014
Background: Corticobasal syndrome (CBS) is characterized by multifaceted motor system dysfunction and cognitive disturbance; distinctive clinical features include limb apraxia and visuospatial dysfunction. Transcranial magnetic stimulation (TMS) has been
A Alberici, A Kertesz, A Kertesz, A Zwinkels, AA Kühn, AK Holl, Alessio Avenanti, B Borroni, B Okuda, BF Boeve, BF Boeve, BP Shelley, C Zadikoff, CD Good, D Claus, D Rueckert, DS Margulies, E De Renzi, E Frasson, E Mioshi, ED Huey, G Goldenberg, G Goldenberg, G Pengas, H Ling, J Ashburner, J Gottlieb, J Rohrer, J Valls-Solé, James R. Burrell, JL Whitwell, JM Cedarbaum, JO Rinne, John R. Hodges, JR Burrell, JR Burrell, JR Burrell, JR Burrell, K Tsuchiya, KA Josephs, KY Haaland, LJ Buxbaum, LJG Rothi, LR Robinson, M Heath, M Hornberger, M Pazzaglia, Matthew C. Kiernan, MC Kiernan, Michael Hornberger, NL Graham, NL Graham, P Soliveri, PK Pal, PP Pramstaller, PS Mathuranath, QJ Almeida, R Cantello, R Chen, R Leiguarda, R Leiguarda, R Mathew, R Mathew, RG Gross, RJ Fisher, RT Watson, S Vucic, S Vucic, S Vucic, S Vucic, SE Lee, SM Smith, SM Smith, Steve Vucic, T Fukui, U Ziemann, V Di Lazzaro, V Stamenova, Y Okuma, Y Zhang   +79 more
core   +3 more sources