progressive bulbar palsy

Introduction

Introduction to medullary progressive paralysis Progressive medullary (ball) paralysis is a degenerative disease of the nucleus of the medullary and cerebral cranial nerves. It is a type of motor neuron disease with progressive dysphagia, unclear articulation, and weakness of the facial and masticatory muscles. The combination of muscle weakness, muscle atrophy and pyramidal tract signs, sensory and sphincter function are generally unaffected. Some patients have a history of chronic heavy metal poisoning. basic knowledge The proportion of illness: 0.0002% Susceptible population: more after the onset of middle age Mode of infection: non-infectious Complications: malnutrition pneumonia

Cause

Medullary progressive paralysis

Cause (40%):

It is a chronic progressive degenerative disease in which the anterior horn cells of the spinal cord, the motor brain of the brainstem group, the cortical pyramidal cells and the pyramidal tract are selectively invaded. The clinical features are the coexistence of impaired symptoms and signs of upper and lower motor neurons.

Prevention

Medullary progressive paralysis prevention

Preventing aspiration To avoid coughing caused by aspiration of food and water, it is forbidden for the patient to eat on his own, to give the indwelling stomach tube, and to carry out nasal feeding. Nasal feeding can be stopped after swallowing experiments have shown that swallowing function is restored. Before pulling out the stomach tube, first inject 30 ~ 50ml of gas into the tube, then fold the tail of the stomach tube and pull it out again. Avoid the food left in the tube being sucked into the trachea during the process of pulling out the stomach tube.

Complication

Medullary progressive paralysis complications Complications

Most patients are malnourished due to dysphagia and are extremely thin. Finally, they often die from asphyxia or aspiration pneumonia. The average survival time after illness is about 2 to 3 years.

Symptom

Medullary progressive paralysis symptoms Common symptoms Dysphagia, pharyngeal reflex, hoarseness, muscle weakness, articulation, lingual muscle atrophy, cough

More than 1 year after the onset of illness, manifested by drinking water cough, difficulty swallowing, chewing, coughing and breathing weakness, dysarthria, examination of the upper palate, pharyngeal reflex disappeared, pharyngeal saliva accumulation, tongue muscle atrophy with muscle bundle Tremor.

2 The cortical medullary bundle is involved in the mandibular reflex, and the late with strong crying and strong laughter, the performance of Zhenzheng and pseudo-paralysis coexist.

3 progress is faster, the prognosis is poor, and more than 1-3 years died of respiratory muscle paralysis and lung infection.

Examine

Medullary progressive paralysis

1. Neuroelectrophysiology: Electromyography is a typical neurogenic change. At rest, the fibrillation potential and positive sharpness can be seen, and sometimes the tremor potential can be seen. When the small force contracts, the motor unit potential time is widened, the amplitude is increased, and the multiphase wave is increased. The vigorous contraction presents a simple phase. The nerve conduction velocity is normal. Motor evoked potentials help to determine upper motor neuron damage.

2, muscle biopsy: helpful for diagnosis, but no specificity, early neurogenic muscle atrophy, late in the light microscopy and myogenic muscle atrophy is not easy to identify.

3, other: blood biochemistry, CSF examination, no abnormalities, creatine phosphokinase (CK) activity can be mildly abnormal, MRI can show that some cases of spinal cord and brain stem atrophy become smaller.

Diagnosis

Diagnosis and differentiation of medullary progressive paralysis

Diagnosis can be based on medical history, clinical symptoms, and laboratory findings.

Differential diagnosis

1. Myasthenia gravis often has medullary symptoms, but no muscle atrophy and fasciculation, the weakness of the affected muscles often manifests as morning light and late, morbid fatigue. If necessary, intramuscular injection of neostigmine can be used as a drug test.

2. Acute infectious polyneuritis is more acute. It often has symmetry weakness in the distal extremities, flaccid paralysis, and protein and cell separation in cerebrospinal fluid.

3. Medullary cavity and brainstem tumors are sometimes similar to this disease, but are accompanied by sensory disturbances.

4. The cerebellopontine angle or the large occipital region of the occipital region often involves the medullary nerve, but it is often one-sided and often has sensory disturbances.

5. Pseudobulbar palsy is a lesion of the bilateral medullary medullary bundle, often a sequela of multiple cerebrovascular accidents, and can also be seen in patients with multiple sclerosis. The clinical manifestations are muscle spasm dominated by the medulla oblongata, but no atrophy of the tongue muscle and fasciculation. The patient's soft palate reflex, pharyngeal wall reflex, and mandibular reflex are active or enhanced, and strong crying and strong laughter can occur.

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