Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an inflammatory central nervous system disorder that mainly involves in the brainstem, basal ganglia and cerebellum. We herein report the case of a patient with CLIPPERS, which was diagnosed based on the clinical and radiological features. After initially responded to steroid treatment, the patient developed limbic encephalitis. The patient presented with memory disturbance, a delirious state and emotional incontinence. A cerebrospinal fluid study revealed interleukin-6 elevation and enhanced bilateral hippocampal lesions were observed on MRI. The patient was successfully treated with methylprednisolone pulse therapy. This is the first case of CLIPPERS with limbic encephalitis involving the bilateral hippocampus.
The amygdala and uncus are located close to important neurovascular structures. We describe a safe technique for resection of amygdala and uncus. Under general anesthesia, the patient is positioned supine, with the head rotated approximately 20 degrees to the unoperated side and slightly extended. By using a trans-anterior T1 subpial approach, the inferior horn of the lateral ventricle is opened, and hippocampectomy is performed. We treat an imaginary plane formed by the inferior circular sulcus of the insula, the endorhinal sulcus, and the inferior choroidal point as the upper border of amygdalar resection. After confirming the position of the inferior choroidal point, the border between the temporal stem and uncus is exposed from anterior to posterior. This border is continuous with the endorhinal sulcus. By exposing the endorhinal sulcus, the anterior choroidal artery and optic tract can be visualized. The amygdala is disconnected through complete exposure of the endorhinal sulcus to the inferior choroidal point. After the lateral side of the uncus is disconnected, the amygdala and uncus are removed en bloc. Since April 2014, we have used the described procedure to remove amygdalar–uncal lesions in 15 patients. The lesion was completely removed in all cases without complications. Histological specimens were obtained in all cases. Our procedure enables safe and complete removal of amygdalar–uncal lesions. Imagining the plane formed by the inferior circular sulcus, inferior choroidal point, and endorhinal sulcus is essential for complete removal of the lesion and for preserving important structures.
Dysembryoplastic neuroepithelial tumor (DNET) is a benign tumor that causes intractable epilepsy at young ages. In this report, we present a rare case of a 28-year-old female with a DNET in the temporal lobe showing extracortical extension to the basal cistern. Enlarging contrast enhancement on MRI, a trait unusual for a benign tumor, suggested the possibility of a malignant glioma. An intraoperative rapid pathological diagnosis of a low-grade glioma in conjunction with electrocorticographic findings supported a surgical strategy of tumor resection alone with preservation of the hippocampus. For this type of DNET with uncommon extension and MRI features, a combination of electro-physiological and morphological analyses based on preoperative studies for epileptogenic focus detection is key to achieving good seizure control and tumor removal with preservation of the patient's memory function.
Since Olds and Miller discovered the space preference phenomenon and related brain structures in 1952, the so-called pleasure center or reward system was identified during animal experiments to test brain function by self-stimulation. Due to advances in brain imaging technology, a large body of evidence now indicates that addiction is a disease of the brain. Thereafter, substance dependence was determined to be closely related with the reward system and parts of the prefrontal cortex. Recent studies in behavioral addiction, such as pathological gambling and internet gaming disorder, demonstrated that the neural substrate for cue-induced craving is similar to that of cue-induced craving in substance dependence. These substrates include the orbitofrontal and dorsolateral prefrontal cortices, the nucleus accumbens, the cingulate cortex, and the caudate nucleus, suggesting that substance and behavioral addiction could share the same neuropathophysiology. Based on this neuroscientific evidence, we developed education materials to prevent addiction. The basic concepts of addiction, including craving, withdrawal and tolerance, loss of impulse control, and social or occupational dysfunction are explained in the material based on results from neuroscience studies In additional, a legislative bill to prevent, manage, and treat addiction was introduced in 2013, regardless of the debate and controversy in Korea. The education material and bill will be helpful to understand and manage patients suffering from addiction.