Acquired Torticollis due to Grisel's Syndrome: Case Report and Follow-Up of Non-Traumatic Atlantoaxial Rotatory Subluxation

Abstract

Non-traumatic atlantoaxial rotatory subluxation is an uncommon entity, with inconsistent presentations. It is also known as Grisel's syndrome (GS), and most commonly follows infectious processes of the upper respiratory tract. A case is described of a non-traumatic rotatory atlantoaxial dislocation in a three-year-old boy. The patient presented with acute torticollis one week after mild upper respiratory infection. Neurological evaluation and lumbar punction were normal. After five days of intractable pain and non-reducible rotational tilt of the head to the left, CT and MR imaging were performed and showed atlantoaxial rotatory subluxation. In addition, MRI demonstrated middle ear and mastoid inflammation. Tilt reduction could be obtained through gentle skull traction under sedation and relaxation for 3 days. Antibiotic treatment was performed. The patient was then placed in a Minerva cast jacket. After seven weeks of immobilization, CT demonstrated regular atlantoaxial alignment. Follow-up after 5.5 years showed the boy neurologically intact and free of clinical complaints. Dynamic cervical radiographs confirmed that the atlantoaxial joints were stable.

Grisel's Syndrome Induced byMycobacterium tuberculosis

“…Grisel's syndrome involves a subluxation of the atlantoaxial joint and sometimes lead to a partial dislocation of the atlantooccipital joint. It usually affects children between 6 and 12 years of age, but may also occur in adults 2 11 14) . Grisel's syndrome was first described in 1930.…”

“…However, it is a rare disease with an apparently low index of suspicion 13) . History taking and clinical signs and symptoms are important clues in the diagnosis of Grisel's syndrome 14) .…”

“…Patients generally seek treatment for progressive throat and neck pain, and pain on attempted motion. One of the outstanding symptoms of Grisel's syndrome is a spontaneously arising, which is the symptomatic manifestation of unilateral or bilateral atlantoaxial subluxation 14) . Neurological complications occur in approximately 15% of these cases and can range from radiculopathy to myelopathy, and may even result in death 4) .…”

“…It is a rare condition, usually seen in children with an upper cervical infection or after an otolaryngeal procedure 2 6 9 11) . Grisel's syndrome is not associated with a trauma or bone disease 14) . Patients generally complain about neck stiffness, pain, and sometimes, difficulty swallowing.…”

“…Grisel's syndrome or "La maladie de Grisel" is a rare non-traumatic atlantoaxial subluxation of the C1-C2 joint, characterized by a spontaneously-occurring unilateral or bilateral misalignment of the atlantoaxial joint 14) . Grisel's syndrome is sometimes associated with subluxation of the atlantooccipital joint 14) . Sir Charles Bell first described a non-traumatic atlantoaxial subluxation in a patient with syphilis, pharyngitis and resultant lethal spinal compression in 1830.…”