Intermittent eye motility disorder with elevation deficiency of one eye

We report about a 12-year-old girl who was referred for neuropediatric evaluation because of an intermittent eye movement disorder. Since 1 year, she had episodes in the morning lasting up to 30 minutes in which she could not look upward with the left eye. During these episodes, the gaze to the side or downward is not impaired. While trying to look upward, she feels a slight pain in the left eye and when the disorder dissolves sometimes a slight click or crackle. Other than these episodes, her eye movements were not impaired and no other neurological symptoms were observed.

Through a short video sequence of the disorder, we describe the diagnosis and possible differential diagnoses. The diagnosis of an acquired Brown syndrome respectively an acquired superior oblique click syndrome was made. In the acquired superior oblique click syndrome, a thickening of the tendon of the superior oblique muscle close to the trochlea leads to a mechanical movement restriction. The thickening traps into the trochlea and this causes the intermittent elevation deficiency, which is most pronounced in adduction. Causes can be systemic diseases (dysthyroid disease, juvenile rheumatoid arthritis, Sjögren syndrome, systemic lupus erythematosus, Hurler-Scheie syndrome) or local orbital pathology (Sinusitis, trochleitis, surgery, orbital fractures). No systemic disease could be shown in our patient. The acquired superior oblique click syndrome can lead to differential diagnosis considerations towards ocular myasthenia, inflammatory central nervous system diseases, vascular diseases or tumors. Therefore, the knowledge of the typical clinical symptomatology of this disorder is important for medical doctors working in the field of pediatric neurology.