Case 39 - Grisel Syndrome, Calcific Tendonitis of Longus Coli,
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I did want to end the spinal segment of the emergency neuroradiology clinical
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scenario with a few other entities. I had mentioned Grisel syndrome as you
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recall previously when I was talking about torticollis rotatory subluxation,
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as well as C1 C2 of atlantoaxial subluxation. Grisel syndrome is secondary
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to an inflammatory process of the pharynx occasionally of the middle ear,
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often involving the retropharyngeal space. And this results in a ligamentous
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laxity that occurs between the C1 and C2 vertebrae. You notice that in
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this example, the patient's space between the odontoid process and the C1
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arches is widened on the left side compared with the right side.
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And this is secondary to this ligamentous laxity that allows torticollis
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to occur. This is some thickening here of the retropharyngeal space.
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Here, you see a different case in which there is rotation of C1
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with respect to the skull base as well as the C2 odontoid process,
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yet another example of Grisel syndrome with torticollis as the presentation.
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Grisel syndrome should not be concerned with another case that we see in
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the emergency department quite frequently and that is calcific tendonitis
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of the longus colli musculature. This is also a case that is very
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frequently shown in boards, as well as in
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maintenance of certification. What one sees is calcification that is embedded
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in the longus colli, longus capitis muscle complex, which is associated
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with retropharyngeal edema. So on the CT scan, we see this retropharyngeal
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space edema as well as the calcification. On the sagittal scan you see
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the edema in low density extending from the calcification all the way down
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to the C5 level with increased space in the interior soft tissues,
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interior to the vertebral bodies. On MR, bright signal intensity from the
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inflammatory process, as well as the low signal intensity of the calcifications
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and you may even see some areas of contrast enhancement in the prevertebral
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musculature, the longus colli, longus capitis muscle complex.
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The importance of this is to recognize that this is not due to
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spinal pathology and an abscess, it's due to the longus colli tendonitis.
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This is treated with nonsteroidal anti inflammatory drugs with response
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in days to weeks, as opposed to an infectious etiology where one would
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require intravenous antibiotics. So no antibiotics necessary, non steroidal
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anti inflammatory drugs and this will resolve over the course of a week.
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I mentioned the entity of Guillain Barré syndrome. You can see in this
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case the diffuse enhancement of the nerve roots
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at the cauda equina, extending onto the surface of the conus medullaris.
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Guillain Barré does not generally show the enhancement in the cord itself.
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This is kind of an autoimmune reaction to the pathogens that are circulating
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in the system and it's an autoimmune reaction unfortunately to the cauda
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equina and nerve roots most commonly. You can occasionally see this in the
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cervical spine nerve roots, much less common than in the lumbar spine nerve
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roots. The differential diagnosis when one has these spinal pathologies
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includes this case. This is a child who has a soft tissue mass
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anterior to the thoracolumbar junction with extension into the paraspinal
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soft tissues. On the axial scan, you see a large mass,
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which is extending into the neural foramen and displacing the spinal cord
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to the left side. With administration of gadolinium, you see the enhancing
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mass as well as the epidural component to this mass. This is not
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an inflammatory process. Were this an inflammatory process, we'd likely
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see with the size of it, some element of necrosis associated with this.
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This is a patient who has a neuroblastoma and in a child it is in
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our differential diagnosis for paraspinal soft tissue inflammation. So this
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is one of the most common of the congenital malignancies. It represents
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30% of all fetal tumors. It's derived from neural crest cells that originate
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from the sympathetic nervous system plexus. You know that with increasing
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age, this converts from neuroblastoma to ganglioneuroblastoma to a ganglioneuroma
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and ganglioneuromas are relatively benign in their course.
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The survival rate is very high and it does respond quite readily to
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chemotherapy.
David M Yousem, MD, MBA
Professor of Radiology, Vice Chairman and Associate Dean
Johns Hopkins University
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