| Introduction: | | | | progressive cervical myelopathy, foramen magnum |
| The Atlantoaxial dislocation (AAD) is a fracture of the | | | | syndrome and sudden death due to compression of |
| odontoid process, in such a way that the end that | | | | vital structures at cervico- medullary junction. |
| forms a joint with the atlas is separated from its | | | | Dystonia is a rare neurological disorder characterized |
| base and kept in position only by the ligaments, which | | | | by sustained muscle contraction with resultant bizarre |
| are not strong, joining it to the atlas. Usually AAD | | | | muscle movements and hence bizarre posturing. |
| presents with occipital pain, others develop vertigo, | | | | Broadly dystonia can be classified as focal, |
| brainstem signs, lower cranial nerve palsies. The | | | | generalized, early onset or late onset. [2] Though |
| brainstem findings occur with either basilar | | | | dystonia due to diverse etiologies like drugs, |
| invaginations or with the alteration of the path of the | | | | degenerative diseases like progressive supra nuclear |
| vertebral artery with changing of normal anatomy. | | | | palsy and even cortical oligoastrocytoma have been |
| Though Idiopathic Cervical Dystonia as a sequel to | | | | described. [3] There has been a frequently reported |
| AAD has been reported in literature. [1] Upper limb | | | | association between peripheral injuries or pain and |
| dystonia is unusual in such cases and has never been | | | | subsequent development of dystonia. Although this |
| reported from India as well as from any part of | | | | has been noted for many years, the mechanism is |
| world and we report a 19-year-old male with AAD | | | | unclear and causative link is speculative. Most cases |
| who presented with limb dystonia and hemiparesis | | | | have been in patients who develop various forms of |
| Case History: | | | | focal adult onset primary torsion dystonia after local |
| A 19-year-old boy born of a non-consanguineous | | | | injuries. Some patients may have pre-existing genetic |
| marriage, following a trivial trauma to the nape of | | | | liability to dystonia but this has been unproven. |
| neck before 2 years developed progressive spastic | | | | Occasionally Dystonia have been described in |
| right hemiparesis. Three months prior to admission, he | | | | surprising clinical settings like spinal cord lesions and |
| developed urinary urgency, precipitancy and | | | | brainstem hemorrhage. [4,5] In the contrary, cervical |
| constipation, and abnormal posturing of right upper | | | | dystonia itself may result in orthopedics and |
| limb predominantly of the hand.There was history of | | | | neurological complications including cervical spine |
| right hemiparesis when he was four years old, which | | | | degeneration, spondylosis, disk herniation, vertebral |
| improved spontaneously over two months. He denied | | | | subluxation and fractures, radiculopathies and |
| history of fever, accident or vaccination prior to the | | | | myelopathy. [6] AAD leading to dystonia could be due |
| present illness. | | | | to multiple factors like limb pain and cervical cord |
| His neurological examination revealed short neck, low | | | | lesion. Our case report is exceptional and AAD leading |
| hairline and spastic right-sided hemiparesis. There was | | | | to limb dystonia has probably never been described in |
| marked hyper-reflexia and clonus with posterior | | | | literature. |
| column impairment without signs of spinothalamic | | | | The exact mechanism of movement disorders in |
| tract involvement. This was associated with | | | | cervical cord lesions is yet not clearly understood. |
| restriction of neck movements and neck spasm. | | | | However, various hypotheses have been proposed |
| There were abnormal movements of right upper limb | | | | which includes altered sensory input, abnormal |
| predominantly distal, in form of repetitive sustained | | | | processing of both input and output signals in the |
| posturing suggestive of dystonia. | | | | spinal interneurons and increased excitability of the |
| The clinical, biochemical and radiological examination | | | | spinal motor neurons. Disruption of the |
| revealed neither evidence of rheumatoid arthritis nor | | | | somatosensory pathways or motor cortex to the |
| any inflammatory, connective tissue disorder. | | | | striatum also may produce abnormal movements |
| Magnetic Resonance Imaging (MRI) of craniovertebral | | | | without sensory loss. |
| junction showed a mobile Atlantoaxial dislocation with | | | | Hand dystonia in our patient was ascribed to AAD |
| a well-developed posterior arch of atlas and the | | | | with cord compression because the abnormal |
| absence of the lamina of the axis.The MRI of brain | | | | movements of hand completely disappeared after |
| was normal. | | | | the correction of AAD. |
| Discussion: | | | | References: |
| Atlantoaxial dislocation (AAD) constitutes an | | | | 1.Kanekar S. Atlantoaxial dislocation in idiopathic |
| important group of Cranio-Vertebral Junction | | | | cervical dystonia. Neurol India 2004 ;52:124-5. |
| anomalies frequently requiring emergency | | | | 2.Jowi JO, Musoke SS.Dystonia: case series of twenty |
| decompression and stabilization of joints to prevent | | | | two patients.East Afr Med J 2005 ;82:463-7. |
| morbidity and mortality resulting from compression of | | | | 3.Koch MW, Luijckx GJ, Leenders KL.Paroxysmal focal |
| neurovascular bundles. Although present since birth, | | | | dystonia with sensory symptoms secondary to |
| patients become symptomatic at a later age (often | | | | cortical oligoastrocytoma.J Neurol2006;253:1227-8. |
| in third decade) usually following a trauma. The | | | | 4.Cammarota A, Gershanik OS, Garcia S, Lera G. |
| trauma may be so trivial, so as to be forgotten by | | | | Cervical dystonia due to spinal cord ependymoma: |
| the patient himself. When present, the severity of | | | | involvement of cervical cord segments in the |
| symptoms and its progression bears no relationship | | | | pathogenesis of dystonia. Mov Disord1995;10:500-3. |
| to the injury sustained. It is suggested that chronic | | | | 5.Esteban Munoz J, Tolosa E, Saiz A, Vila N, Marti MJ, |
| recurrent trauma during neck movements and daily | | | | Blesa R. Upper-limb dystonia secondary to a midbrain |
| activities is an important factor for making the illness | | | | hemorrhage. Mov Disord 1996;11:96-9. |
| symptomatic and its sudden aggravation. Because of | | | | 6.Konrad C, Vollmer-Haase J, Anneken K, Knecht S. |
| its varied clinical presentation and an unpredictable | | | | Orthopedic and neurological complications of cervical |
| course the AAD is often misdiagnosed. Its usual | | | | dystonia- review of the literature. Acta Neurol Scand |
| manifestations include nuchal pain and rigidity, | | | | 2004;109:369-73. |