Cervical (torsion) dystonia or torticollis is disorder characterized by wrong position of the head. It is suspected to be caused by acquired damages in extrapyramidal nervous system and in other cerebral structures leading to unconscious motor function. Neuron disturbances entail hypertonic neck muscles. Among all types of torticollis, ophthalmologists should pay attention to laterocollis manifested through the tilting of the head to one side. A 25-year-old man has been diagnosed with laterocollis for 3 years. The papilledema was more evident with the increased optic nerve disc in the nasal segment, and also optic nerve disc protrusion over the retina. Changes were more evident on the right eye. The temporal sector within the range of 90° remained intact. The moderate decrease of RNFL thickness was defined in the superior and inferior-nasal segments on the right eye, and the low local decrease of RNFL was found in the nasal segment on the left eye.
Cervical (torsion) dystonia (torticollis) is disorder externally manifested wrong position of the head. This pathology is most common in women and debut aged from 20 to 60 years. It is believed that the reason is due to acquired disorders in extrapyramidal nervous system and other cerebral structures, which are generally responsible for unconscious motor function. Irregularities in the neurons lead to an increase in muscle tone of the neck. Clinical manifestations can be varied; in the initial stages are imperceptible, movements of the head smooth, only the patient himself can feel the quiver of the neck muscles. However, visually it is already possible to notice some thickening of the muscle, the person experiences pain at palpation. There is involuntary rotation of the head in one direction or another in the disorder progression. The patient cannot self-correct the position of the head because the neck muscles in these cases are not subject to the will of man. Further, in the pathological process engages more muscles, and change in head position occurs not only in one but in two or even in three directions.
There are several forms of the torticollis depending on the position of the head;
Laterocollis: The head bends to the side and the patient touches his ear with his shoulder
Retrocollis: Backward deflection, head thrown
Antecollis: Chin lowered down
Torticollis: Turn of the head, chin in contact with the shoulder
It was found that the most frequent manifestations of cervical dystonia are muscle pains, as well as various emotional disorders of the personality, psychological problems and social limitations. A high prevalence of anxiety and depressive disorders is known in these patients.
The main method of conservative treatment at present is the use of botulinum toxin preparations, which are injected into intense hypertrophied muscles to block neuromuscular transmission. Injections should be repeated at intervals of 3 to 5 months. In recent years, a more effective method of high-frequency pulsed electrostimulation of structures of the extrapyramidal system of the brain by stereotactic operative access has been used [1].
A 25 year old man was admitted to the Research Center of Neurology for the next course of botulinotherapy, with the diagnosis: Cervical dystonia, 3rd degree of severity and laterocollis to the right. The debut was 3 years ago. Injections of botulinum toxin into the affected muscles and massage of the collar zone are performed with periodicity of 4 to 5 months.
Neuro-ophthalmic status
• Arbitrary eye movements were not observed, the pupil size corresponds to the illumination
• Ptosis of upper eyelid, strabismus, and different width of pupils were absent
• Friendly eye movements were without abnormalities
• Visual acuity was 20/20 without correction
• Intraocular pressure was 12/11 mmHg by pneumotonometry
Computer perimetry
The vision field was normal, the retina photosensitivity was normal. Relative and absolute cattle and the blind spot were not enlarged. There were not pathological changes in the anterior segment by slit lamp microscopy examination.
Ophthalmoscopy and Optical Coherent Tomography (OCT) of the optic disc (Figure 1 & 2): optic nerve disc was pale pink, borders in the nasal, upper and lower regions were blurred. Disc promised anteriorly, stranded above the level of the retina in these areas. The disc diameter was slightly enlarged in the nasal area. The changes were more pronounced on the right eye. The caliber of blood vessels was increased, the fullness of the veins, the ratio of the artery/vein was 1/2, the tortuosity was moderate, the course of the vessels was normal. The macular area and the retina on both eyes were without features, druses and pathological foci.
Figure 1: Optical Coherent Tomography (OCT) of the right peripapillary area.
Figure 2: Optical Coherent Tomography (OCT) of the left peripapillary area.
The OCT conclusion
There were moderate decrease in the thickness of the Retina Nerve Fibers Layer (RNFL) in the superior and superior-nasal segments on the right eye and there were moderate local decrease of RNFL in the superior-nasal segment on the left eye.
The diagnosis
Papilledema of the both eyes in the initial stage (by Tron classification) [2] or I stage (by Frisen classification) [3]. RNFL thickness was decreased in the superior, superior-nasal and nasal segments of the optic nerve region. It was recommended by the neurosurgeon aid.