What causes pseudobulbar palsy?

Pseudobulbar palsy is due to an upper motor lesion caused by bilateral disturbance of the corticobulbar tracts. Pseudobulbar palsy is characterized by dysarthria, dysphagia, facial and tongue weakness, and emotional lability.

What is pseudo bulbar palsy?

Pseudobulbar palsy, also known as involuntary emotional expression disorder, is a condition that affects your ability to control of the muscles in your face (including your jaw). The muscles in your mouth (i.e. your tongue) and your throat can also be affected.

What is bulbar dysarthria?

Bulbar weakness (or bulbar palsy) refers to bilateral impairment of function of the lower cranial nerves IX, X, XI and XII, which occurs due to lower motor neuron lesion either at nuclear or fascicular level in the medulla or from bilateral lesions of the lower cranial nerves outside the brain-stem.

What is the difference between pseudobulbar palsy and bulbar palsy?

Overview. A bulbar palsy is a lower motor neuron lesion of cranial nerves IX, X and XII. A pseudobulbar palsy is an upper motor neuron lesion of cranial nerves IX, X and XII.

What type of dysarthria is associated with pseudobulbar palsy?

Dysarthria due to bilateral disorders of the upper motor neurones may form part of the clinical syndrome of pseudobulbar palsy.

What type of dysarthria is associated with progressive bulbar palsy?

Patients with progressive bulbar palsy initially develop symptoms restricted to the bulbar muscles. They usually present with a several-month history of progressive dysarthria with gagging, choking, and weight loss. The speech disturbance may lead to complete anarthria.

How is pseudobulbar palsy diagnosed?

Diagnosis. Diagnosis of pseudobulbar palsy is based on observation of the symptoms of the condition. Tests examining jaw jerk and gag reflex can also be performed.

What nerve is bulbar palsy?

Bulbar palsy is the result of diseases affecting the lower cranial nerves (VII-XII). A speech deficit occurs due to paralysis or weakness of the muscles of articulation which are supplied by these cranial nerves.

Which cranial nerves are affected in pseudobulbar palsy?

Pseudobulbar palsy is disease affecting the corticobulbar tracts bilaterally. The pathology can affect anywhere along the course of the corticobulbar tracts from the origin in the cortex, to its termination in the cranial nerve nuclei (trigeminal, facial, vagus, hypoglossal and spinal accessory nerves).

What causes Kennedy’s disease?

Causes. Kennedy disease is caused by a change (mutation) in the AR gene that encodes for a protein known as the androgen receptor on the X chromosome.

What type of dysarthria is associated with ALS?

ALS patients usually have a mixed dysarthria (spastic-flaccid). It is characterized by defective articulation, slow laborious speech, imprecise consonant production, marked hypernasality with nasal emission of air during speech and harshness.

How do you treat pseudobulbar palsy?

Dextromethorphan hydrobromide and quinidine sulfate (Nuedexta). This is the only medication approved by the Food and Drug Administration that is designed to specifically treat PBA.

How is bulbar palsy diagnosed?

Other clinical signs associated with bulbar palsy include a nasal speech that lacks in modulation (e.g. controlling or adjusting of one’s speech), difficulty with all consonants, an atrophic (wasting) tongue, drooling, weakness of the jaw and facial muscles, a normal or absent jaw jerk (upward jerk of the jaw upon …

What is the difference between Kennedy’s disease and ALS?

A key feature that distinguishes Kennedy’s disease from ALS is the absence of involvement of upper motor neurons, which causes increased reflexes and spasticity in ALS.

What are symptoms of Kennedy’s disease?

Kennedy disease is typically an adult-onset disease, where symptoms occur mainly between the ages of 20 and 50. The disease is characterized by symptoms such as muscle weakness and cramps in the arms, legs, and facial area, enlarged breasts, and difficulty with speaking and swallowing (dysphagia).

What causes dysarthria in ALS?

Regardless of the site of onset, most patients with ALS will experience bulbar motor deterioration that will lead to a dysarthria. According to Darley’s classification of dysarthrias, dysarthria in ALS is grossly described as “mixed” (both spastic, due the UMN deterioration, and flaccid, due to the LMN deterioration).

What are the first symptoms of bulbar ALS?

Although progression is variable by case, Bulbar Onset ALS tends to have a faster progression than Limb Onset cases. Early symptoms include slurred speech, difficulty chewing and swallowing, excessive choking and weakness or twitching in the muscles of the face, jaw, throat and voice box, particularly the tongue.

Is bulbar palsy ALS?

Progressive bulbar palsy (PBP) is a form of motoneuron disease and is widely classified as a subtype of amyotrophic lateral sclerosis (ALS) with a shorter time of survival and female predominance.

Is Guillain Barre same as ALS?

ALS is a relentlessly progressive, motor-specific illness affecting both the upper and lower motor neurons with fatal outcome. In contrast, MFS and GBS are autoimmune diseases usually preceded by an infection that cause lower motor neuron paralysis with recovery potential.

How long can you live with Kennedy disease?

Treatment is symptomatic and supportive, and life expectancy is normal, though a small percentage of patients (~ 10%) succumb to the disease in their 60’s or 70’s due to swallowing complications (aspiration pneumonia, asphyxiation) resulting from the bulbar weakness.