His bladder and bowel control was still good. His progress was followed up by the orthopaedics outpatient clinic at our centre.ĭuring a clinic visit 2 months later the patient was able to ambulate with support (a walking frame) and power had improved globally to grade 4/5. The family preferred to attend physiotherapy and occupational therapy clinics closer to their home due to travel and treatment costs associated with attending the sessions at KCMC. The patient opted not to undergo surgery as following the 6 weeks on halter traction there was significant return of power to both his upper and lower limbs. Upon discharge, he was also provided with a referral letter for the country’s national hospital, where the definitive treatment of C1–C2 posterior fusion could be carried out. Serial neurological examinations demonstrated improved motor function. The patient was placed on halter traction for 6 weeks in the flexion position with weights of 4kg. Apart from reported paraesthesia in the digits of the right upper limb, the sensory examination was normal (see Table 1). Brisk reflexes were appreciated in the upper and lower limbs and clonus observed at the left ankle. Motor strength in the lower limbs was bilaterally 4/5, both in proximal and distal muscle groups. There was a more profound loss of power in the right upper limb (Proximal: 3/5, Distal: 0–1/5) compared to the left upper limb (proximal: 3–4/5, distal: 3–4/5). Upon examination of the limbs, no other structural abnormalities were appreciated tone was generally increased and a greater degree of loss of motor strength was noted in the upper limbs. The pupils were, however, equal in size, symmetrical and reacted to light. Cranial nerve examination demonstrated ptosis of the right eye with increased sweating on the right side of the face. He had a normal level of consciousness, was quadriparetic and seated upright on a wheelchair with a neck collar. On examination, the patient had typical features of DS consisting of epicanthal folds, flat midface, large protruding tongue, brachycephaly, short puffy fingers and a sandal gap between the first and second toe. Five months prior to the onset of the complaints, the patient was involved in a headlock during a friendly encounter with his playmates, and no other possibly precipitating factors could be recalled. The patient required support for mobility and was otherwise restricted to a wheelchair. Bowel and bladder control was not affected. He also reported episodes of dizziness, numbness on the digits of the right upper limb and excessive sweating. In view of the cervical spine in DS, the sum effect of these factors is decreased stability, with potentially fatal outcomes, following injury due to the proximity of brainstem structures, which centrally control the cardiorespiratory drive as well as the level of the spinal cord (C3–C5) innervating the primary muscle of respiration, the diaphragm.Ī 30-year-old Tanzanian man with previously undiagnosed DS presented with a 5-week history of neck pain radiating to the occiput, along with lower limb weakness 2 weeks following the onset of the pain. The reported incidence of AAI in individuals with DS is recorded as between 6.8 and 27.0% across various reports. El-Khouri’s series of 80 DS patients were assessed for the presence of AAI and 11 (13.7%) individuals had radiographic evidence of the condition, while only one (1.3%) patient had symptoms of cervical instability. This is a well-described developmental anomaly in DS patients, which is defined as an increased (>3 millimetres in adults) distance between the posterior aspect of the frontal arch of C1 and the anterior aspect of the dens of C2 when measured on radiographs taken in the neutral positon. Ligament laxity has been linked with atlanto-axial instability (AAI). Moreover, central hypotonia in DS is one of the neurological characteristics that can be recognized from the neonatal period onwards. Additionally, the connective tissue disorder associated with DS results in ligament laxity, which is linked to joint hyper-flexibility and concomitant instability. Decreases in overall osteoblast activity and lower lifetime peak bone mass values have been linked to the low bone mineral densities found in patients with DS. Studies of the musculoskeletal system in DS patients have demonstrated reduced bone mineral density and ligament laxity. With an estimated incidence of 1 in 1000, Down Syndrome (DS) is one of the most extensively studied chromosomal disorders that is compatible with life.
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