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Pediatric brain stem gliomas with the predominant symptom of sleep apnea
1Department of Otolaryngology, Faculty of Medicine, University of Tokyo, Japan.
Insights
Subtle early symptoms of brain stem gliomas in children, like gait issues and speech changes, can delay diagnosis. Sleep apnea may emerge as a key symptom in later stages of these diffuse brain stem tumors.
Area of Science:
- Pediatric Neurology
- Neuro-oncology
- Respiratory Medicine
Background:
- Brain stem gliomas are primary tumors affecting the central nervous system in children.
- Early diagnosis is crucial for effective management and improved outcomes.
- Subtle or atypical presenting symptoms can lead to diagnostic delays.
Observation:
- Two pediatric cases of brain stem glioma presented initially with subtle neurological deficits.
- Presenting symptoms included unsteady gait, limb weakness, nasality of speech, and subtle sleep apnea.
- Neurological signs were often overlooked or misattributed in the early stages.
Findings:
- Both patients experienced diagnostic delays of several years.
- Magnetic Resonance Imaging (MRI) revealed diffuse, infiltrating lesions in the pons, medulla oblongata, and upper cervical spinal cord.
- Tumor location in the medulla oblongata, crucial for respiratory control, correlated with sleep apnea symptoms.
Implications:
- Delayed diagnosis in pediatric brain stem gliomas can result from subtle, non-specific early symptoms.
- The infiltrative nature of these tumors may contribute to atypical clinical presentations.
- Increased awareness of subtle neurological signs and sleep apnea is needed for earlier detection of brain stem gliomas.
Abstract:
Two children complaining of sleep apnea presented with brain stem gliomas. In the early stage of their illness, neurological disorders were too subtle to be recognized as significant by the physicians or to be noted by the parents. Case 1 experienced an episode of unsteady gait and weakness in the bilateral arms, at the age of 5. When it recurred after 7 years of remission, the predominant symptom was sleep apnea. Case 2 exhibited nasality of speech as the earliest sign of this illness very early in his life, presumably 5 years before the diagnosis of brain stem glioma. A slight sleep apnea which developed afterwards did not draw attention of the physicians because no neurological signs other than paralyses of the bilateral soft palates were present. MRIs of the both cases revealed diffuse, infiltrating lesions in the pons, the medulla oblongata and the upper cervical spinal cord. Both cases shared some features: (1) diagnostic delay of several years from the first symptom; (2) the main lesion in the medulla oblongata, where important structures for respiratory control are identified; (3) infiltrative growth patterns in the MRI of the tumor, which might account for the uncommon clinical courses.