A 10-year-old white boy presented with a 2-year history of staring spells and automatisms followed by 10 to 20 minutes of sleep. He had an unremarkable medical history other than an existing seizure disorder. Medications included divalproex sodium (125 mg, 3 tablets po TID) and levetiracetam (250 mg, 2 tablets po TID). Family history was negative for any central nervous system disorders.
Iman Naseri, MD,
Resident, Otolaryngology Head and Neck Surgery, Emory
University, Atlanta, GA; and
Ranjiv K Saini, MD,
Assistant Professor of Radiology, and
Cynthia T. Welsh, MD,
Assistant Professor of Pathology and Laboratory Medicine,
Medical University of South Carolina, Charleston, SC.
A 10-year-old white boy presented with a 2-year history of
staring spells and automatisms followed by 10 to 20 minutes of
sleep. He had an unremarkable medical history other than an
existing seizure disorder. Medications included divalproex sodium
(125 mg, 3 tablets po TID) and levetiracetam (250 mg, 2 tablets po
TID). Family history was negative for any central nervous system
disorders. The patient was in the fifth grade, lived at home with
his parents, and had two healthy siblings. He was allergic to
lamotrigine, which gave him a generalized rash. His vital signs
were: temperature 97.5¢ª, pulse 91 bpm, blood pressure 124/71 mm
Hg, and weight 39.7 kg. Physical examination revealed no abnormal
findings, and the neurologic examination was normal.
Contrast-enhanced magnetic resonance imaging is the preferred
imaging technique to diagnose ganglio-glioma, as it shows signal
intensity on T1-weighted images, and high intensity on T2-weighted
images. In the images in this case, the tumor presents as a
well-defined cystic mass with a solid mural nodule (Figures 1 and
2). These tumors usually reveal iodine or gado-linium enhancement.
On computed tomography, these tumors are well-circumscribed and
peripherally located. Surrounding edema and hemorrhage is rarely
seen. Most images are hypo- or isodense to brain parenchyma with
poor contrast. Nuclear imaging and angiography are not helpful in
Gangliogliomas are mixed-cell tumors containing both neural
ganglionic and glial cell components. They occur often in patients
<30 years of age and constitute 0.4% to 7.6% of all brain tumors
and approximately 10% of all primary brain tumors in children.
It has been difficult to assign such rare tumors to a particular
category, as they comprise neuronal and glial precursors derived
from the embryonic neuroepithelium. Although they are considered
nonaggressive and are slow-growing, gangliogliomas can cause
long-standing progressive symptoms that include seizures, symptoms
of hydrocephalus, ataxia (if in cerebellum), and headaches.
All imaging techniques are nonspecific, but they aid in the
definition and localization of the tumor. Therefore, diagnosis is
typically established histologically.
This particular patient had a right craniotomy and resection of
the tumor. Histopathology of the brain lesion was found to be a
low-grade ganglioglioma (Figure 3). The prognosis is usually
excellent if these tumors are fully resected.
Although gangliogliomas are rare in the differential diagnosis
of intracranial masses, they should be considered in the presence
of a well-defined cystic mass in the temporal lobes or