they occur most commonly in the thoracic and lumbar areas. Localized pain,
fever, and elevation of inflammatory markers including erythrocyte sedimentation
rate (ESR) and C-reactive protein generally accompany these infections.
Cervical vertebral osteomyelitis may lead to neck stiffness. Vertebral
osteomyelitis is usually bacterial in origin (most commonly caused by S. aureus )
but may be caused by mycobacteria as well. If the cervical spine is involved,
radiographs of this area may reveal destruction of the vertebral body, local soft
tissue swelling, or narrowing of the disc space. MRI is the imaging modality of
choice and may reveal abnormalities of the spine before bony destruction is
visible on plain radiography.
Although uncommon, spinal epidural abscesses are associated with significant
morbidity and mortality. When these abscesses occur in the cervical region,
severe neurologic deficits may occur, and emergent neurosurgical consultation is
essential.
Discitis is uncommon in children; however, cases of cervical discitis have been
reported. This disease is generally seen in children younger than 3 years of age.
The most commonly identified microbiologic organism is S. aureus, although
bacterial cultures are commonly negative and the cause has been debated. When
evaluating for this condition, MRI is indicated.
Generally Non–Life-Threatening Causes
Torticollis due to Minor Irritation, Malposition, and Muscle Spasm. Most
well-appearing children with sudden onset of torticollis without a history of
trauma, fever, or neurologic abnormalities do not have serious underlying
pathology as a cause of their symptoms. Commonly, the patient awakens with
mild neck pain and stiffness. On evaluation, there is no history of trauma, fever,
preceding illness, pharyngotonsillitis, or any additional physical examination
abnormality. The examination reveals a well-appearing child with torticollis,
whose limitation of motion is primarily when he or she attempts correction of the
malposition. Such muscular torticollis may be due to SCM spasm from awkward
sleeping position or other mild irritation. In cases with this type of benign
presentation, nothing more than clinical assessment by history and examination,

analgesic/anti-inflammatory medication, consideration of soft cervical collar, and
close follow-up may be necessary.
Grisel Syndrome. Atlantoaxial subluxation uncommonly may occur as a result
of inflammatory processes in the head and neck region (e.g., rheumatoid arthritis,
systemic lupus erythematosus, tonsillitis, pharyngitis, otitis media,


retropharyngeal abscess) or after otolaryngologic procedures (e.g., tonsillectomy,
adenoidectomy). When due to such infectious or inflammatory conditions, the
subluxation is called Grisel syndrome and is believed to occur as a result of
ligamentous laxity. The subluxation may or may not be associated with
displacement of the atlas, depending on the degree of involvement of the
transverse ligament of the atlas. Most children with Grisel syndrome have
torticollis and neck pain localized to the ipsilateral SCM muscle. Fever and
dysphagia are also common. The child’s head is tilted to one side and rotated to
the side opposite of the facet dislocation. As with rotary atlantoaxial subluxation
from traumatic causes, radiographs may demonstrate the abnormality, but
dynamic CT scan is diagnostic (see previous discussion). In the case of severe
disease, or when there is evidence of spinal cord compression, neurosurgical
consultation is necessary because cervical traction and immobilization are
needed. Grisel syndrome is usually mild, however, and often responds to
analgesic medication, physical therapy, and a soft cervical collar. In addition to
treating the subluxation, antibiotics to treat an underlying bacterial infection may
be needed.
Cervical Lymphadenitis. Cervical lymphadenitis, either acute or chronic, is a
common cause of neck pain and stiffness. The child with this condition typically
has tender swelling over the lateral aspect of the neck, with or without fever. Most
cases of cervical lymphadenitis are caused by S. aureus or group A streptococcus.
Less commonly, mycobacteria and other bacteria including Bartonella henselae,
the cause of cat-scratch disease, may be responsible. Empirical antibiotics to treat

the most common bacterial pathogens are usually sufficient therapy. Screening for
tuberculosis should be performed if any risk factors are present.
Intervertebral Disc Calcification. Intervertebral disc calcification (IDC) is an
uncommon, generally self-limited condition in which the nucleus pulposus of one
or more intervertebral discs calcifies. Both the underlying cause of the condition
and acute symptoms are unknown. Children typically present with 24 to 48 hours
of neck pain associated with neck stiffness or torticollis; fever is often present as
well. The ESR is usually elevated in IDC, and leukocytosis occurs in one-third of
patients. Radiographs of the spine usually show the disc calcification, and CT
scans help localize the calcification within the nucleus pulposus. The calcification
resorbs spontaneously, and the disease is generally benign and self-limited,
although disc protrusion and cord compression may uncommonly occur. One
must distinguish infections of the spine and meningitis (see previous discussion)
from IDC as symptoms and laboratory findings may overlap.


Collagen Vascular Disease. Collagen vascular disease (see Chapter 101
Rheumatologic Emergencies ) may involve the cervical spine and lead to neck
stiffness and/or pain. Children with juvenile idiopathic arthritis (JIA) may have
either insidious or acute onset of symptoms, which commonly include neck
stiffness. Cervical involvement is a late finding in ankylosing spondylitis and
other spondyloarthropathies. However, girls with psoriatic arthritis may have
cervical involvement preceding sacroiliac and lumbar involvement.
Other Infectious/Inflammatory Conditions. Pharyngotonsillitis, upper
respiratory tract infections, otitis media, and mastoiditis may be associated with
neck pain. Torticollis may occasionally be seen with these conditions and may be
accompanied by Grisel syndrome as well. If neck pain is posterior in location and
accompanied by fever, the diagnosis of meningitis should be strongly considered.
The diagnosis of viral myositis involving the neck can be made only after
excluding the possibility of meningitis in a child with neck pain and fever. Upper

lobe pneumonia may cause pain referred to the neck with or without associated
stiffness. Although rare, acute suppurative thyroiditis may cause neck pain and
stiffness and is associated with fever and a palpable, swollen thyroid gland.

Neck Stiffness Associated With Tumors, Vascular Lesions of
the Central Nervous System, or Other Space-Occupying
Lesions
Potentially Life-Threatening Causes
Space-occupying lesions of the brain and spinal cord may lead to neck stiffness,
malposition, and/or pain. Even if the histology of these lesions is benign, they are
potentially life threatening because of the complications of intracranial pressure
elevation and the potential for brain and spinal cord compression. Ruptured
aneurysms may cause subarachnoid hemorrhage with associated neck stiffness.
Brain Tumors. Children with tumors of the posterior fossa, the most common
location for pediatric brain tumors, may present with head tilt, neck stiffness, or
torticollis. Posterior fossa tumors may cause many other symptoms and signs
(e.g., vomiting, headache, ataxia, disturbances in vision including diplopia,
papilledema, cranial nerve deficits, corticospinal or corticobulbar signs). Head tilt
may result from attempts to compensate for diplopia. However, neck stiffness is
believed to result from irritation of the accessory nerve by the cerebellar tonsils
trapped in the occipital foramen or by tonsillar herniation.


Spinal Cord Tumors. Tumors of the spinal cord are uncommon in children and
account for a small fraction of all central nervous system tumors. The most
common spinal cord tumor is an astrocytoma. Typically, spinal cord tumors cause
pain at the tumor site and neurologic defects (e.g., sensory and motor defects,
impaired bowel and bladder function), but symptoms may be slow to develop,
often leading to delays in diagnosis. Spinal cord tumors may also cause torticollis.
Patients with these tumors may also hold their heads in a forward flexed position

(“hanging head sign”). An MRI of the spine should be obtained on any child with
symptoms and signs suggestive of a spinal cord tumor, and emergency
neurosurgical consultation should be obtained.
Vascular Anomalies. Congenital berry aneurysms and acquired cerebral
aneurysms may rupture spontaneously and result in life-threatening subarachnoid
hemorrhages. These can present with abrupt onset of severe headache, neck
stiffness, nausea and vomiting, photophobia, and possibly fever, thus mimicking
meningitis.
Other Space-Occupying Lesions of the Head and Neck. Head and neck tumors
are uncommon in children, and diagnosis requires a high index of suspicion.
Presenting signs and symptoms may include neck pain, stiffness, and/or
torticollis. Rhabdomyosarcomas, Ewing sarcomas, and lymphomas account for
most of the tumors of the neck but other tumors occurring in this region include
nasopharyngeal carcinomas, orbital tumors, acoustic neuromas, osteoblastomas,
and metastatic tumors. Arnold–Chiari malformation of the brain may also cause
neck pain and stiffness.
Other Space-Occupying Lesions of the Spinal Cord. Other uncommon spaceoccupying lesions of the cervical spine such as neurenteric cysts, arteriovenous
malformations, spontaneous spinal epidural hematomas, and syringomyelia may
also cause neck pain and stiffness, generally accompanied by neurologic findings.
Early diagnosis by MRI is essential.
Generally Non–Life-Threatening Causes
Benign Tumors of the Head and Neck. Osteoid osteoma is a benign bone tumor
that commonly affects older children and adolescents. Pain is the typical
presenting symptom, often worse at night. If the osteoma is in the cervical spine,
neck pain and/or stiffness result. Plain radiography is usually diagnostic, showing
a well-demarcated radiolucent lesion surrounded by sclerotic bone. Treatment