Progressive Bulbar Palsy (Fazio Londe Syndrome)

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54 relevant articles (2 outcomes, 3 trials/studies) found for this Disease

Description: 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)

Also Known As:

Fazio Londe Syndrome; Bulbar Palsy; Progressive Bulbar Palsy of Childhood; Syndrome, Fazio-Londe; Bulbar Palsy, Progressive; Childhood Progressive Bulbar Palsy; Bulbar Palsies; Bulbar Palsies, Progressive; Bulbar Paralyses; Bulbar Paralysis; Palsies, Bulbar; Palsies, Progressive Bulbar; Palsy, Bulbar; Palsy, Progressive Bulbar; Progressive Bulbar Palsies; Fazio-Londe Syndrome; Paralysis, Bulbar

Relationship Network

Disease Context: Research Results

Nervous System Diseases: 3422

Neurodegenerative Diseases: 7370

Motor Neuron Disease: 841

Progressive Bulbar Palsy: 54

Neuromuscular Diseases: 744

Motor Neuron Disease: 841

Progressive Bulbar Palsy: 54

Related Diseases

1.	Ophthalmoplegia (External Ophthalmoplegia)
2.	Respiratory Insufficiency (Respiratory Failure)
3.	Syndrome
4.	Amyotrophic Lateral Sclerosis (Lou Gehrig's Disease)
5.	Motor Neuron Disease (Primary Lateral Sclerosis)

Experts

1.	Bilusic, Marijo: 1 article (04/2008)
2.	Pattathil, Jean: 1 article (04/2008)
3.	Zaboski, Michael: 1 article (04/2008)
4.	McHugh, William: 1 article (04/2008)
5.	Brescia, Michael: 1 article (04/2008)
6.	Schanzer, Bernard: 1 article (04/2008)
7.	Kuwabara, Satoshi: 1 article (12/2002)
8.	Mori, Masahiro: 1 article (12/2002)
9.	Yuki, Nobuhiro: 1 article (12/2002)
10.	Koga, Michiaki: 1 article (12/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Progressive Bulbar Palsy:

1.	Intravenous Immunoglobulins (IVIG)FDA Link 12/01/2005 - "Treatment with IVIg resolved the acute ophthalmoplegia, but there was no notable improvement in the AMSAN and bulbar palsy despite repeated" Order ALL the reference details at left...
2.	Immunoglobulin G (IgG)IBA 06/01/2002 - "BACKGROUND: Several patients with GBS and bulbar palsy have been reported to have serum anti-GT1a IgG. Most, however, also have anti-GQ1b IgG. A previous study failed to detect GT1a in human cranial nerves, but GQ1b was abundant in human ocular motor nerves" 06/01/2002 - "All five patients who carried anti-GT1a IgG that did not cross react with GQ1b had bulbar palsy, neck weakness, absence of sensory disturbance, and positive Campylobacter jejuni serology" 06/01/1998 - "The frequency of positive IgG anti-GT1a antibody did not differ in patients with and without bulbar palsy" 11/01/1997 - "The frequency of positive IgG anti-GTla antibody was significantly higher in patients with bulbar palsy than those without bulbar palsy" 04/01/1997 - "These findings suggest that anti-GT1a IgG antibody might play an important role in the pathogenesis of bulbar palsy in GBS." Order ALL the reference details at left...
3.	AntibodiesIBA 06/26/2000 - "In patients who are diagnosed with GBS, the presence or absence of anti-GT1a and anti-GM1b antibodies should be tested at the early stage of GBS so that appropriate therapies that prevent the development of bulbar palsy and improve the outcome of GBS, may be initiated." 11/01/1997 - "We therefore assumed that bulbar palsy was associated with anti-GTla antibodies and made serological study in patients with GBS or Fisher's syndrome" 04/01/2001 - "A case of Guillain-Barré syndrome with bulbar palsy showing the elevations of the anti-GD1a and GT1b antibodies]" 04/01/1999 - "OBJECTIVES: To identify valuable antiganglioside antibodies that support the diagnosis of Guillain-Barré syndrome (GBS) and its variants in patients showing bulbar palsy as an initial symptom" 06/26/2000 - "We examined the serum levels of various IgG antiganglioside antibodies in the sera of 16 patients with GBS with bulbar palsy [GBS-BP(+)] and 72 patients with GBS without bulbar palsy [GBS-BP(-)]" Order ALL the reference details at left...
4.	Insulin-Like Growth Factor I (IGF-1)IBA 12/01/2006 - "In this study, we measured serum and cerebrospinal fluid (CSF) concentrations of GH, IGF-1 and insulin in 35 patients with motor neuron disease (MND) [24 patients with definite amyotrophic lateral sclerosis (ALS) and 11 patients with progressive bulbar palsy] and in 40 healthy controls" Order ALL the reference details at left...
5.	Insulin (Novolin)FDA Link 12/01/2006 - "In this study, we measured serum and cerebrospinal fluid (CSF) concentrations of GH, IGF-1 and insulin in 35 patients with motor neuron disease (MND) [24 patients with definite amyotrophic lateral sclerosis (ALS) and 11 patients with progressive bulbar palsy] and in 40 healthy controls" Order ALL the reference details at left...
6.	Immunoglobulin M (IgM)IBA 05/01/1999 - "The mechanism how the anti-Gal-C IgM antibody induced bulbar paralysis and the symptoms localizing neck and upper limbs remains to be known." 05/01/2001 - "Patients with diarrhea often had anti-ganglioside IgG (anti-GM1 [P=0.0006] and anti-GM1b [P=0.008]), IgM (anti-GM1 [P=0.03], anti-GM1b [P=0.02], and anti-GalNAc-GD1a [P=0.047]) antibodies and rarely showed ophthalmoparesis or bulbar palsy (respectively P=0.02, P=0.04)" Order ALL the reference details at left...
7.	Botulinum Toxins (Botulinum Toxin)IBA 02/28/2001 - "CONCLUSIONS: An aerosolized or foodborne botulinum toxin weapon would cause acute symmetric, descending flaccid paralysis with prominent bulbar palsies such as diplopia, dysarthria, dysphonia, and dysphagia that would typically present 12 to 72 hours after exposure" 04/01/2007 - "To achieve prompt neutralization of circulating botulinum toxin, the decision to treat with Human Botulism Immune Globulin has been based on clinical criteria that include a consistent history and physical findings of bulbar palsies, hypotonia, and weakness" Order ALL the reference details at left...
8.	FlexorIBA 05/01/1999 - "Fifty-five MND patients, clinically divided into 22 amyotrophic lateral sclerosis (ALS), 18 ALS with probable UMN signs (ALS-PUMNS), 10 pure lower motor neuron syndrome (LMNS), and 5 progressive bulbar palsy (PBP), underwent standard TMS, recording from abductor digiti minimi and flexor allucis muscles" 07/01/1999 - "In addition to severe muscle weakness, bulbar palsies, flexor muscle spasm, hypertension, nausea, vomiting, and respiratory compromise were reported or observed" Order ALL the reference details at left...
9.	Triad FDA Link 05/01/2002 - "Botulism is characterized by its classic triad: 1) symmetric descending flaccid paralysis with prominent bulbar palsies 2) afebrile patient 3) clear sensorium" 12/01/2000 - "Infantile botulism classically presents with a triad of clinical features: Bulbar palsies (slow/absent pupil response) Alert Absent fever Other common features are: Constipation, ptosis and poor feeding The diagnosis is a clinical one, confirmed by EMG and by testing stool for the organism, C" Order ALL the reference details at left...
10.	ganglioside M1b (GM1b)IBA 04/01/1999 - "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" 05/01/2001 - "Patients with diarrhea often had anti-ganglioside IgG (anti-GM1 [P=0.0006] and anti-GM1b [P=0.008]), IgM (anti-GM1 [P=0.03], anti-GM1b [P=0.02], and anti-GalNAc-GD1a [P=0.047]) antibodies and rarely showed ophthalmoparesis or bulbar palsy (respectively P=0.02, P=0.04)" Order ALL the reference details at left...

Therapies and Procedures

1.	Enteral Nutrition (Feeding, Tube) 07/01/2007 - "Neurological examination showed facial weakness, lingual atrophy and bulbar palsy, which necessitated the use of a feeding tube and ventilatory support" 02/01/1999 - "Plasmapheresis was performed to relieve respiratory failure in Patient 1 and to shorten the duration of nasogastric tube feeding due to severe bulbar palsy in Patient 2. Significant improvement of electrophysiologic parameters was recorded after plasmapheresis" Order ALL the reference details at left...
2.	Plasmapheresis 02/01/1999 - "Plasmapheresis was performed to relieve respiratory failure in Patient 1 and to shorten the duration of nasogastric tube feeding due to severe bulbar palsy in Patient 2. Significant improvement of electrophysiologic parameters was recorded after plasmapheresis" Order ALL the reference details at left...
3.	Artificial Respiration (Mechanical Ventilation) 04/01/2000 - "She presented with an acute bulbar palsy requiring mechanical ventilation" 04/01/1998 - "However, if dyspnea occurs in the early stage of the disease, when there is no bulbar paralysis and peripheral muscle function is intact, then noninvasive mechanical ventilation via mask may improve the quality of life substantially" 08/01/1994 - "She became abruptly unable to breath following aggravation of bulbar palsy and tetraplegia and mechanical ventilation was immediately initiated" 07/07/1986 - "Bulbar palsy and respiratory failure necessitated endotracheal intubation and mechanical ventilation for five days" Order ALL the reference details at left...
4.	Tracheostomy 04/01/2001 - "The calculated mean survival with NPPV ventilation (criteria: death, tracheostomy, or patient deciding to break off) was 2052 (SE: +/- 317.8) days in the NMD group, 248 days (+/- 35.7) for ALS patients without bulbar symptoms, and 82 days (+/- 27.4) with bulbar paralysis" 09/01/1981 - "She has been left with a severe long-standing bulbar palsy and a permanent tracheostomy" Order ALL the reference details at left...
5.	Oral Medicine 05/01/2002 - "The aim of this report is to show a case of Progressive Bulbar Palsy (PBP), diagnosed by oral medicine specialists, from oral symptoms of the disease" Order ALL the reference details at left...

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