1. Progressive bulbar paralysis is a subtype of amyotrophic lateral sclerosis characterized by atrophy of motor cranial nerve nuclei in the brainstem. 2. It causes weakness of the muscles involved in swallowing and speech leading to drooling, difficulty chewing and talking, and risk of aspiration pneumonia. 3. Symptoms start around age 50-70 and progressively worsen over 1 to 3 years ultimately resulting in respiratory failure and death.

1. Δυσαρθρία
Πιθανή εντόπιση της βλάβης
Ορισμοί :
o Ανώτερος κινητικός νευρώνας
progressive bulbar paralysis o Κατλωτερος κινητικός νευρώνας
o Περιφερκά νεύρα
(medicine) A clinical syndrome due to involvement of the nuclei of o Νωτιαίος μυελός
the last four or five cranial nerves, characterized principally by
paralysis or weakness of the muscles which control :
o swallowing, Μυς που δυνατό συμμετέχουν
o talking,
o movement of the tongue and lips, and o Αναπνευστικοί
o sometimes respiratory paralysis o Άρθρωσης
o Φώνησης
A MOTOR NEURON DISEASE MARKED BY PROGRESSIE o Αντήχησης
WEAKNESS OF THE MUSCLES INNERVATED BY CRANIAL NERVES o Prosody
OF THE LOWER BRAIN STEM.
Clinical manifestations include :
Εξέταση
o dysarthria,
o dysphagia, XII = λέξεις με ‘λ’ =λειτουργία γλώσσας
o facial weakness,
o tongue weakness, and VII = λεξεις με ‘π’ : λειτουργία χειλέων
o 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 Κατώτερο στέλεχος = [progressive] bulbar paralysis
the neuroaxis.
o ΑΤΡΟΦΙΑ / ΔΕΣΜΙΔΩΣΕΙΣ ΓΛΩΣΣΑΣ
Eventually this condition may become indistinguishable from o ΥΠΟΛΕΙΠΟΜΕΝΟ ΑΝΤΑΝΑΓΛΑΣΤΙΚΟ ΕΜΕΤΟΥ
AMYOTROPHIC LATERAL SCLEROSIS. o ΆΝΕΥ ΑΣΤΑΘΕΙΑΣ ?? = NOT LABILE
o ΆΝΕΥ BABINSKI
Fazio-Londe syndrome is an inherited form of this illness which
occurs in children and young adults. (Adams et al., Principles of o ΡΙΝΙΚΗ ΟΜΙΛΙΑ
Neurology, 6th ed, p1091; Brain 1992 Dec;115(Pt 6):1889-1900)
pseudobulbar paralysis
pseudobulbar paralysis o ΘΕΤΙΚΟ BABINSKI
o ΑΥΞΗΜΕΝΟ ΑΝΤΑΝΑΚΛΑΣΤΙΚΟ ΕΜΕΤΟΥ
PARALYSIS OF THE LIPS AND TONGUE, SIMULATING PROGRESSIVE o ΣΥΝΑΙΣΘΗΜΑΤΙΚΗ ΑΣΤΑΘΕΙΑ
BULBAR PARALYSIS, BUT DUE TO SUPRANUCLEAR LESIONS WITH o ΑΝΩΜΑΛΟΣ ΚΥΜΑΤΙΣΜΟΣ ΦΩΝΗΣ = CADENCE
BILATERAL INVOLVEMENT OF THE UPPER MOTOR NEURONS;
characterised by :
άνω κινητικός νευρώνας
o speech and swallowing difficulties,
o emotional instability, and o εκληκτική ομιλία
o spasmodic, mirthless laughter.
παρεγκεφαλίδα
more reading :
o διερευνηυικός λόγος με δυσπροσωδία
http://emedicine.medscape.com/article/317758-overview
εξωπυαραμυδικό
o χαμηλόφωνη ομιλίa

2. Summary of Dysarthrias

3. Terminology Describing Expressive Disorders of Aphasia

4. Summary of Cortical Aphasias

5. PROGRESSIVE BULBAR PARALYSIS
1. Epidemiology
1. Onset age 50-70 years
2. Pathophysiology
1. Subtype of Amyotrophic Lateral Sclerosis (ALS)
2. Cranial Nerve motor nuclear atrophy, glial overgrowth
1. Cranial Nerve 5
2. Cranial Nerve 7
3. Cranial Nerve 9
4. Cranial Nerve 10
5. Cranial Nerve 12
3. Subcortical involvement of corticobulbar tracts
3. Symptoms
1. Drooling
2. Difficult chewing
3. Dysphagia
4. Dysarthria
5. Nasal regurgitation
4. Signs
1. Arm and leg spasticity
2. Hyperactive reflexes
3. Ophthalmoplegia
4. Fasciculation of Tongue and lip muscles
5. Emotional lability
5. Differential Diagnosis
1. Acute bulbar paralysis
1. Acute CNS vascular lesion (hemorrhage or thrombosis)
2. Acute Bulbar Polioencephalitis
2. Chronic bulbar paralysis
1. Syringobulbia
2. Multiple Sclerosis
6. Course
1. Progresses to Aspiration Pneumonia, respiratory arrest
2. Death in 1 to 3 years from onset