Notnagel Syndrome

Notnagel Syndrome is observed in diseases of the quadrigeminal region and is characterized by cerebellar ataxia, paralysis of the III cranial nerve, and deafness; it depends on the involvement of the red nucleus or the brachium conjunctivum, and the nuclei of the III cranial nerve and medial geniculate bodies. It usually begins with symptoms of cerebellar ataxia; then paralysis of the III cranial nerve and deafness are added. In addition to ataxia, intentional tremor, athetoid, and choreiform movements may be observed. The disorder of the III cranial nerve begins with changes in the pupil (dilation, inequality, complete immobility), then develops vertical gaze palsy—inability to move the eyes down and up with free lateral movements; then paralysis of both III cranial nerves; in this case, not all movements are affected equally. The hearing disorder is central deafness, bilateral or unilateral (on the opposite ear). Subsequently—symptoms of paresis or paralysis of the limbs, paralysis of the VII and XII cranial nerves. Among sensory disorders, irritation phenomena have been described.