Pediatric emergency medicine trisk 0974 0974
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Basilar impression is a condition resulting from anomalies at the base of the
skull and vertebrae, which lead to a short neck, headache, neck pain, and cranial
nerve palsies due to compression of the cranial nerves. Many congenital
conditions,
including
Klippel–Feil
syndrome,
achondroplasia,
and
neurofibromatosis, may cause basilar impression. Commonly associated with
basilar impression is occipitocervical synostosis, a condition in which fibrous or
bony connections between the base of the skull and the atlas cause neck pain,
torticollis, high scapula, and neurologic symptoms.
Atlantoaxial Instability. Several congenital conditions may be associated with
atlantoaxial instability and may predispose the patient to cervical subluxation. In
addition to Down, Marfan, and Klippel–Feil syndromes, these include other
skeletal dysplasias and os odontoideum (aplasia or hypoplasia of the odontoid
process of the axis). Morquio syndrome is a mucopolysaccharidosis resulting in
flattening of the vertebrae and multiple skeletal dysplasias. In this syndrome, the
odontoid is underdeveloped and may lead to atlantoaxial subluxation.
Miscellaneous Causes of Neck Stiffness
Head tilt, neck stiffness, and/or torticollis have been reported in other conditions,
some of which are life threatening and others generally benign.
Ophthalmologic, Neurologic, and/or Vestibular Causes. Head tilt or neck
malposition may result from abnormalities of vision (e.g., strabismus, cranial
nerve palsies, extraocular muscle palsies, refractive errors) or the vestibular
apparatus. The child attempts to correct for the disturbance through changes in
neck position. Careful ophthalmologic and neurologic examinations of the child
with head tilt are necessary to exclude these possibilities. Torticollis has also been
reported in patients with migraine headaches.
Myasthenia Gravis. Patients with myasthenia gravis may develop torticollis,
although ptosis, impairment of extraocular muscular movement, and other cranial
nerve palsies are generally earlier signs.
Guillain–Barré Syndrome. Neck stiffness has been reported in children with
Guillain–Barré syndrome. Neck stiffness in this condition is seen in association
with generalized motor weakness and areflexia.
Idiopathic Intracranial Hypertension. Stiff neck and torticollis have also been
reported in children with idiopathic intracranial hypertension, also known as
