Chiari with syrinx

Case #37: MRI: Arnold Chiari II with syrinx

CC: Right sided numbness

Hx: 28 y/o male presented with a 3 month history of progressive right sided numbness; now anesthetic to pain. In addition, he experienced worsening balance, and episodes of aspiration while eating.

PMH:1)Born prematurely and weighed 3#2oz., 2)multiple episodes of aspiration pneumonia as an infant and child, 3) ASD repair age 14, 4)Left ptosis repair age 11, 5) Scoliosis, 6) Gait abnormality, 7) Poor pharyngeal reflexes.

SHx/FHx: mainstream high school education, no mental retardation, ambulatory, works at cardboard shop for the disabled.

EXAM: Short stature. Head tilt to right.

CN: Left ptosis, decreased left nasolabial fold, decreased gag reflex bilaterally.

Motor: full strength

Sensory: marked hypesthesia on entire right side.

Coord: slowed RAM on left.

Station: no drift

Gait: ND

Reflexes: 3+ throughout; Babinski signs bilaterally. 8 beat ankle clonus on right and 3 beat ankle clonus on left.

MRI: Arnold Chiari II with syrinx: Severe basilar invagination, marked compression of ventral pontomedullary junction, downward descension of cerebellar tonsils and vermis.

Course: Patient underwent transpalatal/pharyngeal ventral decompression of pons/medulla with resection of clivus/odontoid and tracheostomy placement. on 9/29/92. Halo vest and ring were removed 6/18/93. 6 months later his Philadelphia collar was removed. He was last seen 4/8/94 and he had mildly spastic gait with good strength and hyperreflexia throughout. His gag response had returned and he was eating without difficulty. Sensation had returned to his extremities.