Clinical Presentation

More than 30% of patients with intracranial lipomas are asymptomatic.23 The vast majority of the remaining patients have signs and symptoms not attributable to the lipoma but secondary to the associated developmental anomalies. Approximately 30% of patients with supratentorial lipomas have a seizure disorder. The seizure disorder is reportedly "often severe, is focal or generalized, and has an average onset age of 15 years. Recurrent headaches are a presenting symptom in 25 percent, and . . . 10 to 15 percent have behavioral problems and/or mental retardation."23 Other reported signs and symptoms include "fainting spells, vomiting, episodic leg weakness, blurred vision, sleepwalking, diencephalic disturbances such as adiposogenital dystrophy and hypothermia, and even transient ischemic attacks."6 Presenting signs and symptoms of patients with infratentorial lipomas may include hydrocephalus, cerebellar ataxia, motor weakness, sleep apnea, and cochleovestibular symptoms such as hearing loss, vertigo, otalgia, trigeminal neuralgia, hemifacial spasm, and tinnitus.6,19,23

The cited incidence of intracranial lipomas ranges from 0.08% to 0.21% of all patients who come to autopsy and 0.06% to 0.30% of all patients studied by CT scans.6,23 However, there are a few autopsy reports that suggest the incidence may be as high as 2.6%.22 Although large MRI series have not been reported, MRI scans might be predicted to yield an even higher overall incidence of lipomas when very small asymptomatic lesions are included. Intracranial lipomas represent 0.1% to 1.3% of all recognized intracranial tumors.6 There have been hundreds of reports of pericallosal lipomas in the world literature since 1818, whereas lipomas of the cerebellopontine angle represent only 0.14% of all tumors seen in this region. A recent report and review of the literature revealed 98 reported cases of lipomas of the CPA or IAC.19

The epidemiology of intracranial lipomas is significant in that there appears to be no age or sex-specific differences with the following exceptions: CPA lipomas are twice as common in males than females, and lipomas in the pediatric population are more commonly localized to the pericallosal cistern.6,19,23 As previously mentioned, several syndromes may also manifest intracranial lipomas, including Bannayan syndrome, an autosomal dominant disorder amenable to genetic counseling; Gorlin-Goldenhar syndrome, encephalocraniocutaneous lipo-matosis, neurofibromatosis variants, and frontonasal dyspla-sia23 (see Table 37-2).

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