Saturday, 27 September 2014

Grisel's Syndrome

Going back to the beginning of Lucy's neck problem I remember her waking with a sore throat and neck one morning, it became better throughout the day. Then a day later it was back again. I took her to the GP who diagnosed tonsilitis.
I wasn't fully happy with the diagnosis at the time because it was obvious that her neck was also bent, I just felt it was more. So I took her to A&E. They said it was torticollis and it would get better in a couple of days. It didn't.
Two weeks and a few more visits to A&E later and she was finally admitted after a CT scan had shown a rotary subluxation of the atlanto occipital joint. 

The consultant was adamant that she had been in an accident and that the subluxation was traumatic. I knew, however, that she hadn't and insisted that it was non-traumatic. Much later I had heard about Grizel's Syndrome and had a conversation by e-mail with an American Neurosurgeon who had suggested Grizel's Syndrome when I'd told him Lucy's story. 

Grizel's syndrome is a rare condition which usually affects children. It occurs more in children who already have lax ligaments, such as those with hypermobility syndrome. It is the subluxation of the atlanto axial joint after an infection such as tonsilitis, Group B streptococuss or similar ear, nose and throat infections. It can also occur after sugery for ENT. The symptoms are torticollis, cervical pain and symptoms related to infection. 

Lucy started with a subluxation of the atlanto occipital joint and was treated with muscle relaxants and soft collar, followed by physio therapy. Three months down the line there was no improvement and in fact the C1 and C2 had subluxed as well. Evidence shows that early treatment and intervention can prevent the situation from getting worse. 

Lucy was eventually given a manipulation and a traction collar, halo, was applied for three months (13 weeks). This did not work. As soon as the halo was removed the subluxation returned and it was discovered that a little piece of the C1 was missing. 

Ten months since the first signs of torticollis Lucy was was given a further manipulation followed by a fixation by Harm's method and the halo re-applied. 

It's been almost fourteen months now and Lucy is free from the halo and soft collar and appears relatively straight again. We will find out next week if her treatment has finally worked.

Her consultant still believes that her original subluxation was traumatic. 

From: Case Reports in Otolaryngology
Volume 2014 (2014), Article ID 703021

In conclusion, diagnosis of the Grisel’s syndrome is largely based on suspicion of the patient who has recently underwent surgery or history of infection in head and neck region. Early diagnosis of the atlantoaxial subluxation is required for careful clinical and radiological evaluation and consultation with relevant branches. Early intervention is critical for prognosis; conversely, delay in diagnosis can be dramatic. Therefore, clinicians should be aware of acute nontraumatic torticollis after recently applying the head and neck surgery 

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