<prompt> All of the following are the classical presentation of Craniovertebral junction anomalies except -Pyramidal signsLow hairlineShort neckPupillary asymmetry  <response>: Craniovertebral anomaliesCraniovertebral anomalies include congenital anomalies at the base of the skull and atlanto occipital region. It includes the occipito atlanto axial complexSymptoms and signs of craniovertebral junction anomaliesSigns and symptoms of craniovertebral junction anomalies reflect the abnormalities and compromise of the following structures.PonsMedullaCervicomedullary junction and upper cervical spinal cordLower cranial nervesCervical nerve rootsVascular supply to the neural structuresIn craniovertebral anomalies, there is involvement of so many neurological structures that it presents with the confusing neurological picture.A patient with congenital anomaly of craniovertebral junction usually presents with an abnormal physical appearance.Congenital anomalies included in craniovertebral anomalies also include Klippel-Feil syndrome and Downs syndrome.Findings in Klippel Feil syndrome is Head cocked to one side Short neckAbnormally low hairline posteriorlyLimitation of neck movementSome more important facts about craniovertebral junction anomaly The most common neurological symptom is posterior occipital headache, that worsens with neck flexion and extension.Myelopathy with involvement of pyramidal tract is also a very common symptom associated with cranio vertebral anomaly.Most common cranial nerve dysfunction is hearing lossPupillary asymmetry usually results due to an abnormality of 2nd or 3rd nerve.The 2nd nerve is cortical and 3rd nerve is related to the midbrain so quite obviously they will not get involved in craniovertebral junctional anomaly. 