Δυσαρθρία

Ορισμοί :

progressive bulbar paralysis

(medicine) A clinical syndrome due to involvement of the nuclei of the last four or five cranial nerves, characterized principally by paralysis or weakness of the muscles which control :

o swallowing, o talking, o movement of the tongue and lips, and o sometimes respiratory paralysis

A MOTOR NEURON DISEASE MARKED BY PROGRESSIE WEAKNESS OF THE MUSCLES INNERVATED BY CRANIAL NERVES OF THE LOWER BRAIN STEM.

Clinical manifestations include :

o dysarthria, o dysphagia,o facial weakness,o tongue weakness, ando 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)

pseudobulbar paralysis

PARALYSIS OF THE LIPS AND TONGUE, SIMULATING PROGRESSIVE BULBAR PARALYSIS, BUT DUE TO SUPRANUCLEAR LESIONS WITH BILATERAL INVOLVEMENT OF THE UPPER MOTOR NEURONS;

characterised by :

o speech and swallowing difficulties, o emotional instability, and o spasmodic, mirthless laughter.

more reading :

http://emedicine.medscape.com/article/317758-overview

Πιθανή εντόπιση της βλάβης

o Ανώτερος κινητικός νευρώναςo Κατλωτερος κινητικός νευρώναςo Περιφερκά νεύραo Νωτιαίος μυελός

Μυς που δυνατό συμμετέχουν

o Αναπνευστικοίo Άρθρωσηςo Φώνησηςo Αντήχησηςo Prosody

Εξέταση

XII = λέξεις με ‘λ’ =λειτουργία γλώσσας

VII = λεξεις με ‘π’ : λειτουργία χειλέων

Κατώτερο στέλεχος = [progressive] bulbar paralysis

o ΑΤΡΟΦΙΑ / ΔΕΣΜΙΔΩΣΕΙΣ ΓΛΩΣΣΑΣo ΥΠΟΛΕΙΠΟΜΕΝΟ ΑΝΤΑΝΑΓΛΑΣΤΙΚΟ ΕΜΕΤΟΥo ΆΝΕΥ ΑΣΤΑΘΕΙΑΣ ?? = NOT LABILEo ΆΝΕΥ BABINSKIo ΡΙΝΙΚΗ ΟΜΙΛΙΑ

pseudobulbar paralysis

o ΘΕΤΙΚΟ BABINSKIo ΑΥΞΗΜΕΝΟ ΑΝΤΑΝΑΚΛΑΣΤΙΚΟ ΕΜΕΤΟΥo ΣΥΝΑΙΣΘΗΜΑΤΙΚΗ ΑΣΤΑΘΕΙΑo ΑΝΩΜΑΛΟΣ ΚΥΜΑΤΙΣΜΟΣ ΦΩΝΗΣ = CADENCE

άνω κινητικός νευρώνας

o εκληκτική ομιλία

παρεγκεφαλίδα

o διερευνηυικός λόγος με δυσπροσωδία

εξωπυαραμυδικό

o χαμηλόφωνη ομιλίa

Summary of Dysarthrias

Terminology Describing Expressive Disorders of Aphasia

Summary of Cortical Aphasias

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 52. Cranial Nerve 73. Cranial Nerve 94. Cranial Nerve 105. Cranial Nerve 12

3. Subcortical involvement of corticobulbar tracts 3. Symptoms

1. Drooling2. Difficult chewing3. Dysphagia4. Dysarthria5. Nasal regurgitation

4. Signs 1. Arm and leg spasticity2. Hyperactive reflexes3. Ophthalmoplegia4. Fasciculation of Tongue and lip muscles5. 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. Syringobulbia2. Multiple Sclerosis

6. Course 1. Progresses to Aspiration Pneumonia, respiratory arrest2. Death in 1 to 3 years from onset