We report a case of reactive arthritis isolated to the tmjs and cervical spine. Having identified this syndrome patient needs to be carefully observed, monitored and treated. Fever and acquired torticollis in hospitalized children the. We present this case report to increase awareness of the condition in the hope that earlier recognition of this disease. Previously there was lack of suitable algorithm for conservative treatment. The neck and head pain usually abates gradually, but the. What links here related changes upload file special pages permanent link page information wikidata item cite this page. The count includes downloads for all files if a work has more than one. Grisels syndrome is characterized by torticollis and a limited range of motion of the neck with pain. Thus, early recognition of the condition with prompt commencement of appropriate conservative treatment could halt the progression into grisels syndrome.
Grisel s diseasec1c2 subluxation2728 drugs sandifer s syndrome. Neurologic problems persist in up to 20 percent of patients with the disease, and onehalf of these patients are severely disabled. Early diagnosis and early treatment are fundamental for a better prognosis of grisels syndrome. Early reduction three hours after trauma and immobilization using only a soft collar were performed and yielded very good. Progressive throat and neck pain and neck stiffness can be followed by neurologic symptoms such as pain or numbness radiating to arms radiculopathies. Grisels syndromea rare complication following small. Grisel syndrome must be included in the differential diagnosis of any patient with torticollis after a head and neck infection, upper respiratory. Subsequent therapy consisted of intravenous meropenem for 4 weeks, followed by oral clindamycin 25 mgkg for 14 days. Reactive arthritis of the temporomandibular joints and. Dec 15, 2008 the differential diagnosis of torticollis includes. A nontraumatic atlantoaxial subluxation thought to be infectious in etiology. Atlantoaxial subluxation is seen after the upper neck. It has rarely been described in a noninfectious acute setting. The clinical files of 12 patients with grisels syndrome were examined retrospectively.
Mri showed a fluid collection just frontal to the clivus with signs of inflammation and signs of c1c2 subluxation. Common paediatric presentations case studies andrew graydon eastwood orthopaedic clinic. A medline search was performed using the terms grisel s syndrome, spontaneous atlantoaxial subluxation, head, neck, ear, nose and throat. Grisel s syndrome gs is a rare condition of uncertain etiology characterized by a nontraumatic atlantoaxial subluxation aas, secondary to an infection in the head and neck region. The objective of this paper is to present a case series of patients with diagnosis of torticollis due to grisel s syndrome and, in doing so, raise awareness of an unusual condition that could be. Dystonia video diagnosis and treatment pichet termsarasab, md steven frucht, md icahn school of medicine at mount sinai new york, ny our work ethic in movement disorders is a video paints a thousand words. We present the case of a 19yearold caucasian woman with traumatic atlantoaxial subluxation. Hellp syndrome is a rare but serious condition that can happen when youre pregnant or right after you have your baby. It is defined as a fixed torticollis combined with a rotatory atlantoaxial subluxation. Grisels syndrome, a rare cause of anomalous head posture. Grisels syndrome designates subluxation of atlantoaxial joint unrelated to trauma or bone disease. In all these cases, there may be a need for stabilization procedures, if there is radiological and clinical requirement. It is a rare condition that occurs almost exclusively in children and has been associated with upper cervical infections and otolaryngologic procedures.
The patients complaints were fully resolved after 2 weeks of conservative treatment. Researchers have identified three types of this disorder, which are distinguished by their genetic cause and pattern of signs and symptoms. A medline search was performed using the terms grisel s syndrome, subluxation. Pdf grisels syndrome is a nontraumatic atlantoaxial subluxation which is usually secondary of an infection or an inflammation at the head and neck. Treatment includes antiinflammatory medications and immobilization of the neck in addition to treatment. It has not been considered, in ophthalmological papers, as a possible cause of ahp. In conclusion, grisels syndrome is a rare complication of upper cervical infections and otolaryngology procedures. Etiopathogenesis has not been clearly described yet, but increased looseness of paraspinal ligament is thought to be responsible. Jan 14, 2020 griscelli syndrome gs is a rare autosomal recessive disorder that results in pigmentary dilution of the skin and the hair silver hair, the presence of large clumps of pigment in hair shafts, and an accumulation of melanosomes in melanocytes. Grisel syndrome torticollis resulting from subluxation of atlantoaxial joint. Atlantoaxial subluxation as an early manifestation in an.
Grisels syndrome is a nontraumatic subluxation of the atlantoaxial joint caused by inflammation of the adjacent tissues. Ear infections and surgical removal of the adenoids can cause an entity known as grisels syndrome, a subluxation of the upper cervical joints, mainly the atlantoaxial joint, due to the inflammatory laxity of the ligaments caused by an infection. Grisel syndrome is a rare cause of torticollis that involves subluxation of atlantoaxial joint from inflammatory ligamentous laxity following an infectious process in the head and neck, usually a retropharyngeal abscess. Grisels syndrome is a nontraumatic subluxation of the atlantoaxial joint caused by.
Odontoid subluxation syringomyelia and vertebral artery abnormalities 90 int j clin exp med 2019. Here the authors report a case of a malian 5yearold girl with grisels syndrome following tonsillectomy. In this case, definitive diagnosis was only made via mri as ct could not identify presence of infection. Three variants of griscelli syndrome have been identified. Grisel s syndrome is nontraumatic atlantoaxial subluxation aas secondary to an inflammatory process in the upper neck. Torticollis may occur in congenital and acquired forms. She has lifted the bandages from the 400 yearold abscess of slavery that. Only papers focusing on true nontraumatic atlantoaxial subluxation were included. Griscelli syndrome type 1 involves severe problems with brain function in addition to the distinctive skin and hair coloring.
Increased antenatal visits and deliveries attended by skilled health care providers following the introduction of antenatal ultrasound in rural uganda. There are few references to this syndrome in the ent literature but it may occur in association with. This is a rare disease that usually affects children. Grisel syndrome resulting from a retropharyngeal abscess or osteomyelitis of c2 was suspected. Atlantoaxial instability has been described as a manifestation of ankylosing spondylitis juvenile and adult onset, reactive arthritis, juvenile idiopathic arthritis, and rheumatoid arthritis. Grisel syndrome definition of grisel syndrome by medical. Hellp stands for the different things that happen when you have it. Grisels syndrome is a nontraumatic atlantoaxial subluxation which is usually secondary of an infection or an inflammation at the head and neck region.
The patients complaints were fully resolved and the patient gained full cervical spine range of motion after 1 week of treatment. A rare complication following traditional uvulectomy article pdf available in pan african medical journal 20 june 2015 with 155 reads how we measure reads. When the inflammation heals, this can probably result in a fixation in the rotated position. Case series of both adult and pediatric cases neck. Grisel syndrome is nontraumatic atlantoaxial subluxation, resulting from inflammatory ligamentous laxity following an infectious process in the head and neck. In the usa, 1525% of trisomy 21 patients are diagnosed with atlantoaxial dysfunction 35. To avoid this, we recommend that torticollis be described in terms of the direction of both head and ear tilt and chin rotation eg, torticollis with head tilt to the left and chin rotation to the right. Upper respiratory tract infections are the commonest cause. Apr 14, 2010 the atlantoaxial subluxation grisels syndrome is a rare complication following operative procedures andor infections in the upper aerodigestive tract. Atlantoaxial rotatory fixation a cause of torticollis. Jan 30, 2005 grisels syndrome, defined as subluxation of the atlantoaxial joint, not associated with trauma or bone disease, is found primarily in children. The 1st and 2nd joints were fully debonding with rimer through fora. Traumatic atlantoaxial rotatory subluxation in an adolescent. Grisels syndrome is a nontraumatic atlantoaxial subluxation which is.
Torticollis is not a diagnosis, but it is a sign of underlying disease. Grisel syndrome following meningitis and anaerobic bacteremi. Temporomandibular joint tmj arthritis is frequently seen in children with chronic arthritis. Any other pathology such as marfan syndrome and grisels syndrome may also cause instability 9. It can be observed after surgery of head and neck region. A 6yearold native american boy hospitalized for treatment of lymphadenitis and aseptic meningitis had an incidental brain magnetic resonance imaging mri. Affected individuals typically have delayed development, intellectual disability, seizures, weak muscle tone hypotonia, and eye and vision abnormalities. Presence of torticollis following otolaryngological surgery upper respiratory infections should arise suspicion of grisel syndrome 4. A list of lyrics, artists and songs that contain the term grisels syndrome from the lyrics. Mar 01, 2016 anomalous head posture ahp or torticollis is a relatively common condition in children. Case series of both adult and pediatric cases were included. Information and translations of grisels syndrome in the most comprehensive dictionary definitions resource on the web.
Grisels syndrome is a nontraumatic atlantoaxial subluxation associated with. The patient often has moderate pain when the head is kept motionless, while movement towards a normal position triggers pain. Pathogenetically the higher flexibility of the ligaments during the inflammation causes a subluxation between axis and atlas. These patients may present earlier in the disease process without evident subluxation. Carrier screening to help detect the risk of having a baby with a specific inherited disorder, such as cystic fibrosis. A case of aas secondary to an upper cervical infection is presented. Grisels syndrome causes doctor answers on healthcaremagic. Grisels syndrome gs is a rare condition of uncertain etiology characterized by a nontraumatic atlantoaxial subluxation aas, secondary to an infection in the head and neck region. Clinical outcomes of posterior c1 and c2 screwrod fixation.
Mar 11, 2020 fulltext downloads displays the total number of times this works files e. Grisel s syndrome is a rare entity and usually develops in paediatric patients after otolaryngologic surgery or infection. Evaluation of persistent torticollis following adenoidectomy. Despite delayed presentation, we successfully reduced it consevatively via halter traction. Late diagnosis and inadequate treatment may cause neurological. Usually, the treatment of choice is traction using halogardnerwells fixation devices for up to six weeks. Grisel s syndrome is a common cause of nontraumatic aarf.
A 7yearold girl developed severe neck stiffness and trismus while on treatment for a presumed throat infection. American society of emergency radiology annual meeting. Jan 23, 2012 atlantoaxial rotatory subluxation is rarely caused by trauma in adults. These nodes also drain the paranasal sinuses, the nasopharynx and retropharyngeal area.
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