Clinical diagnosis-part I: what is really caused by Chiari I.
Palma CiaramitaroMarilena FerrarisFulvio MassaroDiego GarbossaPublished in: Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery (2019)
Herniation of the cerebellar tonsils may lead to significant clinical symptoms, including neck and cervical pain, short-lasting occipital "cough" headache, dizziness, and gait impairment; in children younger than 3 years, oropharyngeal symptoms are prevalent (sleep apnea, feeding problems) whereas in those older than 3 years, a higher incidence of cough headache and scoliosis is reported. CM1 clinical features, both in children and in adults, have in common the presence of anatomical deformities of the brainstem and cerebellum. Clinical myelopathy (sensory/autonomic disorders, motor weakness) can result from direct compression of the cervical spinal cord by the herniated cerebellar tonsils or can be due to the presence of a syrinx, reported in association with Chiari I between 35 and 75% of pediatric patients. Similarly, in our series (440 females, 160 males, 98% > 18 years), syringomyelia associated with Chiari I was ranging from 40 to 60% (respectively in asymptomatic and symptomatic groups); headache was reported in 65%. Sensory disturbances (48%), cranial nerve deficits (45%), motor weakness (32%), and autonomic disorders (35%) were the most frequent neurological signs in our cohort. In Chiari I malformation, cervical pain and occipital cough headache are the most characteristic presenting symptoms, both in old children and in adults; however, headache is often multifactorial, and CM1 patients can report a wide variety of non-specific symptoms and signs. Clinical diagnostic CM1 criteria, shared at the national and international level, are recommended with the aim to avoid consequent controversies on diagnosis and on surgical decision making.
Keyphrases
- spinal cord
- sleep apnea
- young adults
- chronic pain
- neuropathic pain
- end stage renal disease
- traumatic brain injury
- spinal cord injury
- pain management
- obstructive sleep apnea
- mental health
- heart rate variability
- chronic kidney disease
- risk factors
- peritoneal dialysis
- newly diagnosed
- heart rate
- physical activity
- quality improvement
- community dwelling
- blood pressure