PubMed · 7445151
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J C Krarup, O Kristensen. 1980-09-15. [Progressive supranuclear paralysis].. https://pubmed.ncbi.nlm.nih.gov/7445151/
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A 5-year-old female was evaluated because of severe speech and expressive language delay. On examination, she could hardly speak and communicated through gestures. She manifested severe dysarthria and difficulty in protruding and moving her tongue laterally. She lacked coordination of the swallowing process, with drooling and an increased mental reflex. Her cognitive development was normal, and no associated neurologic dysfunction of the limbs was noted. On follow-up, the child experienced two episodes of seizures at 6 years of age. Magnetic resonance imaging of the brain demonstrated perisylvian and frontal polymicrogyria. Clinical and radiologic findings demonstrated a similarity and continuum between congenital suprabulbar paresis (Worster-Drought syndrome) and perisylvian syndrome.
Bulbar dysfunction resulting from corticobulbar pathway or brainstem neuron degeneration is one of the most important clinical problems encountered in motor neuron disease (MND) and contributes to various respiratory complications which are major causes of morbidity and mortality. Chronic malnutrition as a consequence of bulbar muscle weakness may have a considerable bearing on respiratory muscle function and survival. Abnormalities of the control and strength of the laryngeal and pharyngeal muscles may cause upper airway obstruction increasing resistance to airflow. Bulbar muscle weakness prevents adequate peak cough flows to clear airway debris. Dysphagia can lead to aspiration of microorganisms, food and liquids and hence pneumonia. MND patients with bulbar involvement commonly display an abnormal respiratory pattern during swallow characterized by inspiration after swallow, prolonged swallow apnoea and multiple swallows per bolus. Volitional respiratory function tests such as forced vital capacity can be inaccurate in patients with bulbofacial weakness and/or impaired volitional respiratory control. Bulbar muscle weakness with abundant secretions may increase the risk of aspiration and make successful non-invasive assisted ventilation more difficult. We conclude that an evaluation of bulbar dysfunction is an essential element in the assessment of respiratory dysfunction in MND.
We describe a patient presenting with progressive bulbar dysfunction and spasticity that clinically mimicked amyotrophic lateral sclerosis (ALS). Electromyography, however, showed no evidence of denervation and revealed a rare combination of peripheral and central myokymia. We feel that this pattern of myokymia represented a marker of neural injury from remote radiation therapy. Nervous system disorders resulting from therapeutic radiation are described, and potential pathophysiologic mechanisms underlying myokymia are discussed.