All of the following are the classical presentation of Cranioverebral junction anomalies except -
Ans. is 'd' i.e., Pupillary asymmetry Cranioveebral anomalies Cranioveebral anomalies include congenital anomalies at the base of the skull and atlantooccipital region. It includes the occipito atlanto axial complex Symptoms and signs of cranioveebral junction anomalies Signs and symptoms of cranioveebral junction anomalies reflect the abnormalities and compromise of the following structures. a) Pons b) Medulla c) Cervicomedullary junction and upper cervical spinal cord d) Lower cranial nerves e) Cervical nerve roots f) Vascular supply to the neural structures o In cranioveebral anomalies there is involvement of so many neurological structures that it presents with confusing neurological picture. o A patient with congenital anomaly of cranioveebral junction usually presents with an abnormal physical appearance. Congenital anomalies included in cranioveebral anomalies also include Klippel-Feil syndrome and Down's syndrome. o Findings in Klippel -- Feil syndrome is Head cocked to one side Sho neck Abnormally low hairline posteriorly Limitation of neck movement Some more impoant facts about cranioveebral junction anomaly o Most common neurological symptom is posterior occipital headache, that worsens with neck flexion and extension. o Myelopathy with involvement of pyramidal tract is also very common symptom associated with cranioveebral anomaly. o Most common cranial nerve dysfunction is hearing loss o Pupillary asymmetry usually results due to abnormality of 2"d or 3rd nerve. o The 2"d nerve is coical and 3rd nerve is related to the mid brain so quiet obviously they will not get involved in cranioveebral junctional anomaly.
Pyramidal signs
Low hairline
Sho neck
Pupillary asymmetry