Noonan syndrome is a relatively common multiple congenital abnormalities syndrome first described by Noonan and Ehmke in 1963 and further characterized by Noonan in 1968. It is considered an autosomal dominant trait with an incidence of 111,000 to 112,500 live births. It is a syndrome of short stature, webbed neck, hypertelorism, downward slant of palpebral fissures, ptosis, apparently low-set ears, small mandible, low posterior hairline, cubitus valgus, short fifth finger with clinodactyly, wide-spaced nipples, sternallverte-bra1 deformities, and frequent cardiovascular anomalies. The cardiac anomalies, vascular abnormalities, and coagulation deficiencies are well-known. Numerous neurologic abnormalities have also been described in individual cases including cases of cerebrovascular defects. However, to my knowledge, brain infarcts have not been reported in the Noonan syndrome.
CLINICAL REPORT
C.W. is a 15-year-old white girl who was an unrestrained passenger in a motor vehicle accident and suffered a mild traumatic brain injury. Initial head CT and neck radiographs at a local hospital were interpreted as normal, and a scalp laceration was sutured. She experienced nausea, vomiting, and an occipital headache, was admitted for 24-hour observation, and, subsequently, discharged home. C.W. was readmitted 2 days later complaining of right-sided weakness, some difficulty speaking, and right facial numbness. A repeat CT showed questionable infarcts in the right pons. The patient's right-sided symptoms resolved within several hours. However, she developed left-sided symptoms of weakness, decreased coordination, and numbness shortly thereafter. MRI of the head (Fig. 1) demonstrated multiple brainstem infarcts, including lesions in the pons (right more severe than left), right cerebellum, and questionable lesions in the temporal lobes.