One and a Half Syndrome

This 44 year old woman presented in 1973 with an acute attack of optic neuritis in the right eye that fully recovered after a course of ACTH therapy. In 1991, 18 years later, she developed unsteadiness of gait, "walking like a chicken", stiff legs that jerked spontaneously in bed at night, and numbness in the legs distal to the knees. The left leg was more affected than the right. She had urinary frequency, urgency and occasional incontinence. She denied any symptoms in the upper extremities. She had a negative L'Hermitte symptom. In hot weather her legs became weak and fatigue increased (Uhthoff's symptom). Past History: Negative for additional neurological symptoms Family History: Negative for CNS disease. Neuro-ophthalmological examination: Visual acuity OD: 20/25, OS: 20/20 Right afferent pupil defect Mild dyschromatopsia OD Fundus exam showed optic atrophy OD. Normal optic disc OS Ocular Motility A left horizontal gaze palsy with gaze evoked nystagmus A left internuclear ophthalmoplegia on gaze right with adduction weakness OS Abducting nystagmus OD Full vertical gaze Upbeat nystagmus on upgaze (ill sustained) Convergence normal Neurological examination: Titubation Spastic paraparesis, with spastic left foot drop Marked hyperreflexia, bilateral ankle clonus and extensor plantar responses. Ataxia on finger-nose and heel-knee-shin Broad base spastic ataxic gait. Spinal tap: Cerebrospinal fluid protein 51 mg/dl, sugar 66 mg/dl, 4 white blood cells, elevated IgG and positive oligoclonal bands. VDRL negative. Brain MRI: MRI of the brain with gadolinium showed multiple white matter lesions in the central and periventricular white matter. The largest of these lesions was approximately 9 mm in diameter and ring enhancing, reflecting the presence of both acute and chronic demyelinating lesions consistent with a diagnosis of MS. MRI study of the cervical spine showed cervical spondylosis with disc herniation at C5-6 and C6-7 lateralizing to the right and compressing the spinal cord. A neurosurgeon recommended conservative management. Diagnosis: Multiple Sclerosis (MS) Treatment: The patient received high dose intravenous methylprednisolone 1,000 mg in 1,000 cc of dextrose saline daily for 5 days, then tapered to a dose of 500 mg. in 500 cc of dextrose saline daily for 2 days. Only the patient's gait improved on treatment. See also: http://content.lib.utah.edu/cdm/ref/collection/ehsl-shw/id/346