There are different types of clinical presentations, including progressive bulbar palsy (PBP), Limb-onset ALS, progressive muscular atrophy (PMA) and upper motor neuron (UMN) predominant ALS. PBP has mainly brainstem signs. [cambridge.org]

We present a case of sarcoidosis with a unique presentation of isolated bulbar palsy. [cureus.com]

Auditory involvement is usually not present in progressive bulbar palsy. [accessanesthesiology.mhmedical.com]

Both bulbar and pseudobulbar palsy are seen mainly in men over 75 years old and present with dysarthria and dysphagia. [amboss.com]

Nasal regurgitation may be present. Dysphonic. Dysphagic. Emotional lability may also be present. There may also be neurological deficits in the limb - eg, increased tone, enhanced reflexes and weakness. [patient.info]

• Acute Intermittent Porphyria

  Causes[edit] Genetic: Kennedy's disease, acute intermittent porphyria Vascular causes: medullary infarction, such as lateral or medial medullary infarction. [en.wikipedia.org]

  Causes Genetic: Kennedy's disease, acute intermittent porphyria Vascular causes: medullary infarction Degenerative diseases: amyotrophic lateral sclerosis, syringobulbia Inflammatory/infective: Guillain–Barré syndrome, poliomyelitis, Lyme disease Malignancy [m.blog.naver.com]

  The authors present a case of acute intermittent porphyria (AIP) in an almost fatal relapse with quadriplegia, bulbar paralysis and coma [3]. [wikigenes.org]

  Användning i kommersiellt syfte är förbjuden. sv dc.subject lääketiede fi dc.title Neurological manifestations and molecular genetics of acute intermittent porphyria in North Western Russia en dc.type.ontasot Doctoral dissertation (article-based) en dc.type.ontasot [helda.helsinki.fi]

• Constipation

  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 [wikigenes.org]

  Autonomic neuropathy manifested as abdominal pain (88%), tachycardia (94%), hypertension (75%) and constipation (88%). [helda.helsinki.fi]

• Abdominal Pain

  Autonomic neuropathy manifested as abdominal pain (88%), tachycardia (94%), hypertension (75%) and constipation (88%). [helda.helsinki.fi]

• Tachycardia

  Autonomic neuropathy manifested as abdominal pain (88%), tachycardia (94%), hypertension (75%) and constipation (88%). [helda.helsinki.fi]

• Muscular Atrophy

  […] lateral sclerosis G12.24 Familial motor neuron disease G12.25 Progressive spinal muscle atrophy G12.29 Other motor neuron disease G12.8 Other spinal muscular atrophies and related syndromes G12.9 Spinal muscular atrophy, unspecified G13 Systemic atrophies [icd10data.com]

  Learn about this topic in these articles: subtypes of amyotrophic lateral sclerosis In amyotrophic lateral sclerosis: Course of the disease …are progressive muscular atrophy and progressive bulbar palsy. [britannica.com]

  Progressive proximal spinal and bulbar muscular atrophy of late onset. A sex-linked recessive trait. Neurology. 1968 Jul. 18(7):671-80. [Medline]. Finsterer J. Bulbar and spinal muscular atrophy (Kennedy's disease): a review. [medscape.com]

  atrophy (PMA) or Duchenne-Aran muscular atrophy extremely rare; childhood (Fazio-Londe disease presents between 2-12yrs, and those aged 1-5yrs may present with stridor) and adult forms; 4% of MND; similar features to ALS but lacks UMN features and has [ozemedicine.com]

  (Adams et al., Principles of Neurology, 6th ed, p1091; Brain 1992 Dec;115(Pt 6):1889-1900) progressive muscular atrophy pseudobulbar palsy + spinal muscular atrophy + Synonyms Exact Synonyms: Bulbar Palsy ; Bulbar Paralyses ; Bulbar Paralysis ; Progressive [rgd.mcw.edu]

• Myopathy

  Discussion: Thyrotoxicosis may commonly result in skeletal proximal myopathy, however rarely it may cause marked wasting of the bulbar muscles resulting in dysphagia, dysphonia and dysarthria. [endocrine-abstracts.org]

  Dropped head syndrome can be seen in inflammatory myopathies, myasthenia gravis, facioscapulohumeral muscular dystrophy, spinal muscular atrophy, nemaline myopathy and carnitine deficiency (Umapathy et al, 2003) but ALS should also be considered in patients [cambridge.org]

  Sarcoidosis may present with a wide array of presentations such peripheral neuropathy, myopathy, mono neuritis multiplex, Guillain barre like syndrome, bilateral facial nerve palsy, and last but not least as leptomeningitis while the parenchymal spread [cureus.com]

• Suggestibility

  Suggested Listings for Evaluation: DETERMINATION LISTING REMARKS Meets 11.17 11.22 111.17 111.22 Equals * Adjudicators may, at their discretion, use the Medical Evidence of Record or the listings suggested to evaluate the claim. [secure.ssa.gov]

  The NCS was suggestive of demyelinating sensorimotor polyneuropathy. His CSF showed albumin-cytological dissociation and the serological testing was positive for IgM anti-GM2 antibodies. [neurologyindia.com]

  In addition to axonopathy, features suggestive of demyelination could be demonstrated in patients with severe PNP during an acute attack. PNP with a moderate muscle weakness was mainly pure axonal. [helda.helsinki.fi]

  They suggested that other antibodies were more important (e.g. anti GQ1b). Hashiguchi et al (1997) reported Guillain-Barre with anti-GT1a. Lametery et al (2016) reported PCB -- pharyngeal-cervical brachial syndrome, a bulbar palsy, without ataxia. [dizziness-and-balance.com]

  Cranial magnetic resonance imaging suggested lower cranial nerve involvement. Serum anti-GM1 IgG and anti-GD1b IgG antibodies were positive. After stopping aripiprazole, his bulbar symptoms improved. [pedneur.com]

• Fear

  Inability to move or function; total stoppage or severe impairment of activity: fear that led to national paralysis. [thefreedictionary.com]

• Withdrawn

  […] for ALS may include: laboratory tests - including blood and urine studies and thyroid functioning tests muscle and/or nerve biopsy cerebral spinal fluid analysis (spinal tap) - a procedure used to make an evaluation or diagnosis by examining the fluid withdrawn [hopkinsmedicine.org]

• Nasal Speech

  Signs of Bulbar Palsy Absent jaw jerk Absent palate rise Absent pharyngeal reflex Tongue wasting and fasciculations Flaccid dysarthria - nasal speech with impaired articulation and shortened phrases Causes of Bulbar Palsy Motor neurone disease Guillain-barré [medschool.co]

  [edit] nasal speech lacking in modulation and difficulty with all consonants tongue is atrophic and shows fasciculations. dribbling of saliva. weakness of the soft palate, examined by asking the patient to say aah. normal or absent jaw jerk absent gag [en.wikipedia.org]

  Signs & Symptoms Symptoms dysphagia (difficulty in swallowing) difficulty in chewing nasal regurgitation slurring of speech difficulty in handling secretions choking on liquids dysphonia (defective use of the voice, inability to produce sound due to laryngeal [m.blog.naver.com]

  speech emotions are not affected pseudobulbar palsy normal or increased reflexes - gag reflex and jaw jerk spastic weakness - small, tight tongue that cannot be protruded “Donald Duck” speech labile emotions bilateral upper motor limb signs other differential [ozemedicine.com]

• Quadriplegia

  […] body part or a disturbance in the functioning of a social group; "erectile dysfunction"; "sexual relationship dysfunction" paresis - a slight or partial paralysis paraplegia - paralysis of the lower half of the body (most often as a result of trauma) quadriplegia [thefreedictionary.com]

  The authors present a case of acute intermittent porphyria (AIP) in an almost fatal relapse with quadriplegia, bulbar paralysis and coma [3]. [wikigenes.org]

• Seizure

  Differential Diagnosis Alcohol withdrawal seizure and delirium tremens[8] Metabolic encephalopathy[7] Pathological laughter also correlates with large compressive posterior fossa tumors, posterior fossa meningiomas, epidermoid tumors, trochlear nerve [statpearls.com]

  Screening for acute porphyria using urinary PBG is useful in a selected group of neurological patients with acute PNP or encephalopathy and seizures associated with pain and dysautonomia. [helda.helsinki.fi]

• Akinesia

  ThesaurusAntonymsRelated WordsSynonymsLegend: Noun 1. paralysis - loss of the ability to move a body part akinesia, akinesis - motionlessness attributable to a temporary paralysis alalia - paralysis of the vocal cords resulting in an inability to speak [thefreedictionary.com]

• Neurologic Manifestation

  Neurological symptoms during an acute attack, which is the major manifestation of AIP, are variable and relatively rare, but may endanger a patient's life. [helda.helsinki.fi]

Workup for poliovirus was negative. Daily physiotherapy was started under supervision. [pediatricneurosciences.com]

Packard Center for ALS Research at John Hopkins offers further general information on treatment: http://www.alscenter.org/what-is-als/treatment/index.html The Mayo Clinic provides information on treatment of ALS in general, which may be helpful: http: [rarediseases.info.nih.gov]

Bulbar palsy and pseudobulbar palsy Summary Epidemiology Subtypes and variants Diagnostics Treatment [amboss.com]

Pisa syndrome during aripiprazole treatment: a case report. [pedneur.com]

As there is no cure for Bulbar Palsy, treatment is essentially limited to medical management and support. [newyorkfacialparalysis.com]

Neurology. 1966; 16: 212-216 Elliot JL Hudson AJ Treatment of communication disorders. in: Mulder DW The diagnosis and treatment of amyotrophic lateral sclerosis. [oooojournal.net]

IBP patients appear to have a relatively benign prognosis and longer survival compared to PBP.[sup][5],[6] It is important to distinguish IBP from PBP for prognosis prediction, patient care, and even treatment options. [go.gale.com]

Clinical features and prognosis of Miller Fisher syndrome. Neurology. 2001; 56: 1104-1106 Uysalol M. Tatlı B. Uzel N. Çıtak A. Aygün E. Kayaoğlu S. [pedneur.com]

Prognosis This depends on the underlying cause. [patient.info]

ICD-9: 335.22 ICD-10: G12.22 PROGRESSION The prognosis for PBP is poor. The symptoms of PBP slowly worsen with onset beginning between the ages of 50-70 years. [secure.ssa.gov]

Because the condition is degenerative, the prognosis for PBP is fairly poor. [disability-benefits-help.org]

CSF analysis and MRI of the brain help identify the etiology. Treatment is mainly supportive. Epidemiology References:[1][2] Epidemiological data refers to the US, unless otherwise specified. [amboss.com]

Etiology Pseudobulbar palsy is not a disease but a clinical constellation. Clinical conditions that involve corticobulbar tracts bilaterally can cause pseudobulbar palsy.[3] Many pathological conditions can lead to pseudobulbar palsy. [statpearls.com]

Although much has been learned about the etiologic factors involved in the acute forms of bulbar paralysis,2 anterior poliomyelitis and associated diseases, the cause of true progressive bulbar palsy is just as obscure today as when the condition was [jamanetwork.com]

Epidemiology References:[1][2] Epidemiological data refers to the US, unless otherwise specified. [amboss.com]

[…] subsets]. 61 Pradat PF...Bruneteau G 17128087 2006 32 Masseter spasticity successfully treated with neuroablations of the bilateral mandibular nerves for a patient with progressive bulbar palsy. 61 Fujiwara Y...Shimada Y 16116026 2005 33 Clinical and epidemiological [malacards.org]

He has completed fellowship training in both intensive care medicine and emergency medicine, as well as post-graduate training in biochemistry, clinical toxicology, clinical epidemiology, and health professional education. [litfl.com]

Pseudobulbar palsy epidemiology depends on the cause that has lead to corticobulbar pathway damage. [statpearls.com]

Epidemiology of the post-polio syndrome. Am J Epidemiol. 1992 Oct 1. 136(7):769-86. [Medline]. Takemura J, Saeki S, Hachisuka K, Aritome K. Prevalence of post-polio syndrome based on a cross-sectional survey in Kitakyushu, Japan. [medscape.com]

Nervous system disorders resulting from therapeutic radiation are described, and potential pathophysiologic mechanisms underlying myokymia are discussed. (C) 2000 John Wiley and Sons, Inc. [mayoclinic.pure.elsevier.com]

Infectious neuropathies: poliomyelitis, diphtheria, neurosyphilis Stroke (multiple, recurrent strokes) Neurodegenerative disease: amyotrophic lateral sclerosis, progressive supranuclear palsy, multiple sclerosis Injury or malignancy of the high brainstem Pathophysiology [amboss.com]

Amelioration of the underlying disease process Prevention of complications Improvement of swallowing via therapy Compensations to improve swallowing safety and efficiency & 5. [slideshare.net]

Physical therapy can help the patient to keep their muscles functional and prevent some muscle spasms. Another option is a medication, which includes especially anti-depressant (against emotial lability) or some analgesics. [wikilectures.eu]

These affected nerves become damaged, preventing the messages from the nerve reaching the muscles causing weakness and muscle wasting. [southtees.nhs.uk]

Postural changes can help with drooling of saliva and may prevent aspiration. [patient.info]

Our case highlights the importance of considering sarcoidosis as a possible cause of bulbar palsy; timely efforts are needed to confirm the diagnosis and treat the condition to prevent any permanent neurological damage. [cureus.com]