Introduction
Hopkins Syndrome (HS) represents an exceedingly rare neurological disorder characterized by the abrupt onset of acute flaccid paralysis (AFP) typically subsequent to an exacerbation of asthma. The condition predominantly affects the pediatric population and poses significant diagnostic and therapeutic complexities due to its infrequency and an incompletely understood pathophysiology.
Historical Perspective
- Initial Description: First delineated by Hopkins et al. in 1974.
- Initially associated with status asthmaticus, though later cases linked it to milder asthma exacerbations.
- Often conceptualized as a variant of post-viral or post-infectious myelitis, exhibiting distinctive clinical features that set it apart.
Epidemiology
- Age of Onset: Primarily affects children, particularly those under the age of 10.
- Incidence: Extremely rare, with most data derived from isolated case reports or small series.
- Gender Disparity: No significant sex predilection has been established.
Pathophysiology
- Asthmatic Association: A consistent temporal relationship between asthma exacerbations and the onset of paralysis.
- Viral Triggers: Hypothesized involvement of respiratory viruses such as Enterovirus, Coxsackievirus, and Adenovirus, though no definitive causative agent has been identified.
- Autoimmune Hypothesis: Immune-mediated destruction of anterior horn cells in the spinal cord, potentially triggered by asthma-related immune dysregulation.
- Hypoxic Contribution: Hypoxia during severe asthma episodes may exacerbate neuronal injury, although the exact mechanism remains speculative.