A case of unilateral cerebellar agenesia

Abstract

TWENTY FIGURESThe brain in this case came into the hands of the writer in the course of collecting some brains for anatomical purposes. The clinical symptoms were not thoroughly studied and recorded, but the following details were procured some time after from the physician in charge in reply to a letter of inquiry by Prof. M.Allen Starr.She was a small child, her head was small and she was very weak and unsteady in all her movements. Shc could walk, but with a very uncertain gait, staggering to one side (which side not noted) and with a tendency to hold fast to chairs, etc., in getting about. She could move her head but the movements were slow. No paralysis was noted. She had marked bilateral nystagmus, exact type not noted.She was very weak mentally, appeared very dull, took little interest in toys, etc. She talked poorly and indistinctly, with scarcely any formed sentences. No convulsions nor spasms were noted, nor vomiting except during her. last illness. It was not possible to be sure whether she had headache, hut for many weeks after. admission to the institution of which she was an inmate she cried almost constantly and rolled her head from side to side. Aft,er this crying ceased she almost continuously during the (lay hummed, without words, a few tunes, one popular air of the t h e especially. This humming was so marked, so continuous during the day, as to be a 'ward joke'-and accompanying the music, a nevei ceasing rotation of thc head from side to side, usually in time with the music, and evcntually resulting in depriving the back of the head of hair.The child, a girl, was 3 years, 4 months, 16 days old.She sat up all day in a high-backed chair.She was unsteady in grasping a proffrrctl object.Whether she was deaf in either ear was not noted.Death was due to measles and broncho-pneumonia. The brain was fixed in situ by intravascular injection with formalin not long after death and was consequently n excellent condition both as regards histological fixation and freedom from

Outcome of severe unilateral cerebellar hypoplasia

“…In almost all of the patients a brainstem asymmetry was easily recognizable. This asymmetry included hypoplasia of the ipsilateral middle and superior cerebellar peduncles and superior colliculus, a highly asymmetrical pons with absent contralateral olivary body, and contralateral reduction of the red nucleus and the substantia nigra, as comprehensively reported by Strong, 17 and these are considered to be secondary changes.…”

“…DISCUSSION UCH was first described by Strong in the postmortem examination of a 3-year-old female with learning disability. 17 Since then additional pathological reports of UCH have been published. 18,19 The advent of computed tomography and MRI has facilitated the recognition of this lesion in vivo.…”

Crossed Pontine Hemiatrophy Associated with Unilateral Cerebellar Hemorrhage in Premature Infants

“…5 The first description of the effects of a unilateral cerebellar lesion on the contralateral pons dates back to 1915. 6 More recently, the contralateral brainstem asymmetry was shown in patients with unilateral cerebellar hypoplasia. 7,8 CBH is an increasingly recognized cause of cerebellar hypoplasia, especially among extremely preterm infants.…”