From the Guidelines
Progressive bulbar palsy patients with severe dysphagia should undergo gastrostomy feeding to provide long-term enteral nutrition therapy, as it may improve survival, quality of life, and nutritional outcome, as suggested by the study of McDermott et al. 1.
Overview of Progressive Bulbar Palsy
Progressive bulbar palsy is a motor neuron disease characterized by degeneration of lower motor neurons in the brainstem, affecting muscles involved in speech, swallowing, and facial expressions. Treatment focuses on symptom management as there is no cure.
Symptom Management
Riluzole (50mg twice daily) is the primary medication, which may extend survival by 2-3 months by reducing glutamate excitotoxicity. For symptom management, anticholinergics like glycopyrrolate (1-2mg three times daily) can reduce excessive saliva, while baclofen (5-25mg three times daily) or tizanidine (2-8mg three times daily) help with spasticity.
Nutritional Support
Nutritional support is crucial, often requiring a percutaneous endoscopic gastrostomy (PEG) tube when swallowing difficulties become severe, as recommended by the ESPEN guideline on clinical nutrition in neurology 1. The study by McDermott et al. 1 suggests that gastrostomy feeding can prevent weight loss in half of the patients and lead to weight gain in 25% of them.
Respiratory Support and Multidisciplinary Approach
Respiratory support, including non-invasive ventilation at night, becomes necessary as respiratory muscles weaken. A multidisciplinary approach involving neurologists, speech therapists, nutritionists, and palliative care specialists is essential for comprehensive management. Early discussion about end-of-life care preferences is important as the disease progresses, typically over 2-3 years.
Key Considerations for Gastrostomy
The timing of gastrostomy insertion is crucial, and the study by McDermott et al. 1 suggests that patients might benefit from early gastrostomy, i.e., before substantial weight loss that might not be reversible has occurred. The relation between respiratory function and risk of mortality and outcome after gastrostomy is still a matter of controversy, but the study by Spataro 1 suggests that survival in patients with PEG is not affected by the severity of the respiratory impairment.
- Key points to consider:
- Gastrostomy feeding can improve survival, quality of life, and nutritional outcome in patients with severe dysphagia.
- Early gastrostomy insertion may be beneficial before substantial weight loss occurs.
- Respiratory function may not be a significant factor in determining the outcome after gastrostomy.
- A multidisciplinary approach is essential for comprehensive management of progressive bulbar palsy.
From the FDA Drug Label
The ALSFRS-R scale consists of 12 questions that evaluate the fine motor, gross motor, bulbar, and respiratory function of patients with ALS (speech, salivation, swallowing, handwriting, cutting food, dressing/hygiene, turning in bed, walking, climbing stairs, dyspnea, orthopnea, and respiratory insufficiency) The decline in ALSFRS-R scores from baseline was significantly less in the edaravone injection-treated patients as compared to placebo
The edaravone injection is used to treat people with amyotrophic lateral sclerosis (ALS). The drug label does not specifically address progressive bulbar palsy. However, it does mention that the ALSFRS-R scale evaluates bulbar function, which includes speech, salivation, and swallowing.
- The study showed that edaravone injection slowed the decline in ALSFRS-R scores compared to placebo.
- The ALSFRS-R scale assesses the functional ability of patients with ALS, including bulbar function.
- However, the label does not provide direct information on the treatment of progressive bulbar palsy with edaravone injection 2 2.
From the Research
Definition and Classification of Progressive Bulbar Palsy
- 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 3.
Clinical Course and Survival
- The median survival time for patients with PBP is approximately 35-40 months, with almost all patients progressing to ALS regardless of the presence of upper motor signs or generalized denervation on EMG of the limbs 4.
- Rapid physical decline at the end of life is associated with bronchopulmonary infection and excessive oral secretion, leading to a high level of symptom burden 3.
Treatment and Management
- Riluzole is the only treatment that prolongs ALS survival, and its efficacy is present amongst bulbar-onset ALS and older patients, but not in subjects with limb-onset 5.
- Riluzole therapy increases survival rates at 12 months by approximately 10% and prolongs survival by 6 months 5.
- Edaravone has been used through intravenous mode to halt the progression of ALS, and it shows efficacy for managing ALS and increasing the life span of patients 6.