PMON118 Cerebral Palsy Resulting in Hypothalamus Volume Loss and Temperature Dysregulation

BACKGROUND: Cerebral palsy is a syndrome caused by a non-progressive brain injury or malformation during early brain development, which can lead to severe physical disability. However, there is limited data on the consequences of cerebral palsy on other crucial brain functions such as hypothalamus and pituitary gland. We report a case of hypothalamic-pituitary involvement in a cerebral palsy patient. CLINICAL CASE: A 28-year-old man with history of quadriplegic cerebral palsy, severe cognitive delay and seizure disorder presented to our clinic with temperature dysregulation for 2 years. He is wheel-chair-bound and non-verbal. His core temperature generally ranges from 96.8o to 97.2oF but occasionally he has had fluctuations as high as 102oF and as low as 88oF associated with extremes in surrounding temperature. At the time of initial evaluation, his temperature was 96.8oF (36°C) and his skin was cool and mottled. He was on levothyroxine replacement for hypothyroidism and thyroid hormone levels were within the normal range (Free T4 of 1.33 ng/dL and Total T3 of 153 ng/dL). Head CT revealed extensive encephalomalacia involving the frontal and temporal lobes, diffuse thinning of the parenchyma of the parietal and occipital lobes with secondary lateral ventricle enlargement, and volume loss in the deep gray nuclei including the hypothalamus. Further evaluation revealed a borderline response to Cortrosyn (16.9 ug/dL at 60 min) but associated with an adequate baseline cortisol of 13.7 ug/dL. IGF-1 and prolactin levels were normal (IGF-1 of 117 ng/mL and prolactin of 7.9 ng/mL), but testosterone was 175 ng/dL associated with an LH of 3.1 mIU/mL and FSH of 10.7 mIU/mL consistent with central hypogonadism. The family was advised to carefully regulate and monitor ambient temperature, and maintain dress appropriate for the surrounding temperature. CONCLUSION: Although periventricular white matter lesions are most commonly associated with cerebral palsy resulting in motor dysfunction, deep gray matter lesions are also occasionally documented. These lesions should be recognized, as they could be detrimental to the body. The hypothalamus, with the preoptic area as its main thermoregulatory locus, plays an important role in temperature regulation. It integrates all information from the surface thermoreceptors and local brain temperature then initiates heat production or heat loss mechanisms to help maintain homeostasis. Therefore, volume loss of the hypothalamus can lead to serious consequences such as poikilothermia that can lead to life-threatening hypothermia or hyperthermia. Keywords: Cerebral palsy, hypothalamus, temperature dysregulation, poikilothermia Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.