Type 1 split cord malformation
This article describes the case of a previously healthy 60-year-old female of Flijan descent who presented with complaints of lower limb pain, weakness and a decline in mobility. Doctors initially diagnosed the patient with hip osteoarthritis and ligamental knee injury. She was later referred to a neurologist who admitted her to a tertiary care hospital with new-onset upper motor neuron signs and urinary incontinence. Magnetic resonance imaging of the spine was done which showed type 1 split cord malformation (SCM) for which the patient required urgent spinal surgery.
According to the patient, the pain started 6 months ago with progressive lower limb weakness and left lateral thigh pain. Her mobility was severely impacted. She used to be a community ambulator and was now relying on a wheelchair for mobility over the course of 6 months. Her medical history revealed the diagnosis of spina bifida at birth which required surgical repair in infancy. The surgical procedure left behind no signs of residual deficits. Doctors further advised an MRI of the spine which led to the diagnosis SCM.
Initially her symptoms were managed the same as those of degenerative osteoarthritis
However, because the patient’s symptoms progressed, she visited the emergency department where she was suspected of having knee ligament injury. Orthopaedic surgeons reviewed the case, ruled out the diagnosis and referred him to a neurologist. The patient’s symptoms continued to worsen with worsening mobility, urinary incontinence and onset of spasticity. She was admitted urgently for further investigations.
Test results showed an unremarkable upper limb and truncal examination with signs of weakness in the lower limbs, bilateral ankle clonus, spasticity in the left leg and hyperreflexia. The MRI showed evidence of partial fusion of the vertebral bodies with hydromyelia at the level of T2 and T3. In addition to type 1 SCM at C7-T2 with spina bifida.
Treatment included C6-T1 laminectomy with decompression of the intramedullary cyst at the level of T1. After the procedure there was significant improvement in her lower limb strength. Whereas she made consistent neurological and functional gain over the next 3 weeks, after her transfer to the rehabilitation ward. Pain and spasticity in her leg also reduced significantly. In addition, she gained unaided community ambulation just after 4 weeks of outpatient physiotherapy.
Source: Journal of Neurosurgery