Grisel syndrome is nontraumatic atlantoaxial subluxation, resulting from inflammatory ligamentous laxity following an infectious process in the head and neck. Grisels syndrome that is known as the subluxation of atlas and axis was firstly described by sir charles bell in 1830 in a syphilitic patient with pharyngeal ulceration. Guleryuz a, bagdatoglu c, duce mn, talas du, celikbas h, koksel t. 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. Upper respiratory tract infections are the commonest cause.
Grisel s syndrome has been described in the otolaryngologic, neurosurgical, and orthopedic literature as a rare consequence of inflammatory, infectious andor postsurgical complication in the head an. Grisels syndrome is a nontraumatic atlantoaxial subluxation which is usually secondary of an infection or an inflammation at the head and neck region. The importance of early diagnosis and appropriate treatment in grisels syndrome. Grisels syndrome is a nontraumatic subluxation of the atlantoaxial joint caused by inflammation of the adjacent tissues. We present a case of atlantoaxial subluxation occurring in a 20year. Grisels syndrome the lymphatic drainages of the upper cervical joints are primarily into the retropharyngeal lymphatic nodes and then into the deep cervical chain. Early diagnosis of grisels syndrome in children with. Griscelli syndrome type 1 involves severe problems with.
Although first described in 1830, the exact mechanism of grisel s syndrome remains unclear. Anomalous head posture ahp or torticollis is a relatively common condition in children. Grisels syndrome is a rare but welldocumented clinical entity. In conelusion, grisels syndrome is a rare elinicalentity that may be seen in a variety ofelinical settings. This is a rare disease that usually affects children. Griscelli syndrome is an inherited condition characterized by unusually light hypopigmented skin and light silverygray hair starting in infancy.
Grisel syndrome may remain undiagnosed at initial presentation if not suspected. The patients complaints were fully resolved after 2 weeks of conservative treatment. Grisels syndrome is a rare but dangerous complication that can go unnoticed in its early phase and can be a major cause of morbidity and mortality following infection or head and neck. Bell reported death in this patient by spinal cord compression due to atlantoaxial subluxation and autopsy report showed the erosion of the axis transverse ligament 1. Three variants of griscelli syndrome have been identified. Grisels syndrome is a rare condition of uncertain aetiology characterized by atlantoaxial subluxation following an infection in the head and neck region. Bell reported death in his patient by spinal cord compression due to atlantoaxial subluxation and autopsy report showed the erosion of the axis transverse ligament 11, 12. Etiopathogenesis has not been clearly described yet, but increased looseness of paraspinal ligament is thought to be responsible. Pdf grisel syndrome is defined as atlantoaxial subluxation following infections of upper airway. It is known as an inflammatory atlantoaxial rotatory subluxation following upper respiratory tract infections urti and surgical otolaryngological procedures. Cervical magnetic resonance imaging mri allows prompt and safe diagnosis, and a threedimensional computed tomography ct scan provides better classification of the instability. Information and translations of grisels syndrome in the most comprehensive dictionary definitions resource on the web. Grisel syndrome must be included in the differential diagnosis of any patient with torticollis after a head and neck infection, upper respiratory.
People with gs2 have unusually light skin and silvercolored hair. Grisel s syndrome uncountable english wikipedia has an article on. Torticollis is not a diagnosis, but it is a sign of underlying disease. The point to be stressed is that prompt, individualized treatment is essential for full functional recovery. Conservative treatment in grisels syndrome following. In extreme cases, the condition can lead to quadriplegia and.
In children, when unresponsive neck rigidity and distress are observed after ear, nose and throat ent surgical treatment or nasopharyngeal inflammation, grisels syndrome should be suspected. Oct 25, 2018 the painful torticollis only itself may be a major sign for the diagnosis of grisels syndrome gs. This is a rare syndrome involving nontraumatic rotatory subluxation of the atlantoaxial joint. 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. Septic arthritis of the c1a c2 lateral facet joint and. Grisel s syndrome known as the subluxation of atlas and axis was firstly described by sir charles bell in 1830 in a syphilitic patient with pharyngeal ulceration. We report a 5 year old boy with recurring episodes of head tilt and painful and restricted neck movements that developed after repeated bouts of sinusitis.
In children with painful torticollis following ent procedures or nasopharyngeal inflammation, grisels syndrome should always be suspected. Grisels syndrome occurs in children and is the concurrence of lateral atlantoaxial subluxation and torticollis, caused by localized infection of adjacent. Grisels syndrome, a rare cause of anomalous head posture in. The concurrence of nontraumatic atlantoaxial subluxation with inflammation of the adjacent neck tissues is known as grisels syndrome. Grisels syndrome has been described in the otolaryngologic, neurosurgical, and orthopedic literature as a rare consequence of inflammatory, infectious andor postsurgical complication in the head an. Grisels syndromea rare complication following small operations and infections in the ent region.
The condition most frequently affects children and characteristically presents in the postoperative period with cervical pain and torticollis. A rare complication following adenotonsillectomy abdulkadirbucak, 1 sahinulu, 1 abdullahaycicek, 1 emrekacar, 2 andmuratcemmiman 3 department of otolaryngology, faculty of medicine, afyon kocatepe university, ali cetinkaya kampusu. Grisel syndrome torticollis resulting from subluxation of atlantoaxial joint. These nodes also drain the paranasal sinuses, the nasopharynx and retropharyngeal area. Nontuberculous bacterial in volvement of the clc2 lateral facet joints with tor ticollis has not been reported. Although first described in 1830, the exact mechanism of grisels syndrome remains unclear. Le journal canadien des sciences neurologiques, year2009, volume36 1. It is a nontraumatic, fixed rotary subluxation of c1 on c2 atlantoaxial. Grisel s syndrome is a rare but welldocumented clinical entity. Griscelli syndrome type 2 gs2 is a rare, inherited condition that affects the skin, hair, and immune system.
It can be observed after surgery of head and neck region. Atlantoaxial subluxation grisels syndrome following. In conelusion, grisel s syndrome is a rare elinicalentity that may be seen in a variety ofelinical settings. Leong department of otolaryngology, liverpool university hospitals and alder hey childrens hospital, liverpool, uk published in international journal of pediatric otolaryngology 2007. Every otolaryngologistshould be famiiiar with its epidemiology, presentation. May 01, 2017 griscelli syndrome type 2 gs2 is a rare, inherited condition that affects the skin, hair, and immune system. 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 is a rare entity characterized by nontraumatic atlantoaxial rotatory subluxation, which usually occurs after infections of head and neck or after otolaryngology procedures such as adenoidectomy, tonsillectomy, and repair of choanal atresia, among others. The painful torticollis only itself may be a major sign for the diagnosis of grisels syndrome gs. 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. Researchers have identified three types of this disorder, which are distinguished by their genetic cause and pattern of signs and symptoms. Progressive throat and neck pain and neck stiffness can be followed by neurologic symptoms such as pain or numbness radiating to arms radiculopathies.
Grisels synd rome that is known as the subluxation of atlas and axis was firstly described by sir charles bell in 1830 in a syphilitic patient with pharyngeal ulceration. Grisels syndrome, a rare cause of anomalous head posture in children. Grisel s syndrome is a rare condition of uncertain aetiology characterized by atlantoaxial subluxation following an infection in the head and neck region. Suml\lajry two cases of grisels disease located below the atlantoaxial joint, one of them being of multiple location, are reported. Mar 24, 2014 grisels syndrome that is known as the subluxation of atlas and axis was firstly described by sir charles bell in 1830 in a syphilitic patient with pharyngeal ulceration. Bell reported death in this patient by spinal cord compression due to atlantoaxial subluxation and autopsy report showed the erosion of the axis transverse ligament 1 3. 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. Griscelli syndrome type 2 genetic and rare diseases.
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