Clinical Features and Surgical Management of Cerebellopontine Angle Cholesteatoma That Presented as Trigeminal Neuralgia.

BACKGROUND: It is difficult to differentiate patients with cerebellopontine angle (CPA) cholesteatoma and patients with primary trigeminal neuralgia just according to early symptoms. We aimed to explore the clinical characteristics, early diagnosis, and microneurosurgical techniques for CPA cholesteatoma that presented as trigeminal neuralgia. METHODS: The data of 26 patients who complained trigeminal neuralgia with CPA cholesteatoma between January 2009 and December 2015 were collected and studied retrospectively, they were diagnosed by magnetic resonance imaging preoperatively and confirmed by pathology postoperatively. All the tumors were resected through a retrosigmoidal approach. In 26 cases, 14 patients who underwent cholesteatoma resection and microvascular decompression were assigned to group A and 12 patients who underwent only cholesteatoma resection were assigned to group B. The clinical features and surgical results between groups A and B were compared. The complications and surgical results were followed up, and surgical techniques were summarized. RESULTS: All patients presented as trigeminal neuralgia at the same side of the cholesteatoma. There was no statistical difference between the 2 groups in clinical features and surgical results between groups A and B. All patients with cholesteatoma showed clear and significant imaging characteristics. The tumors were totally removed in 18 patients and subtotally removed in 8 patients. Pain relief was satisfactory in all patients. Surgical complications included transient aseptic meningitis in 2 patients, facial numbness in 2 patients, mild tinnitus in 2 patients, mild and facial weakness in 1 patient. No death, hematoma, or acute hydrocephalus were reported in this series. During the follow-up of 12-80 months, no patient experienced recurrence of pain or tumor. CONCLUSIONS: Cholesteatoma of the cerebellopontine angle often presented as trigeminal neuralgia. Magnetic resonance imaging is helpful for early diagnosis according to its distinct signal. Surgical treatment is often the first choice, the neuralgia relief was satisfactory after operation. Microvascular decompression is recommended simultaneously if some offending vessels were founded during the surgical resection of the tumor.

Long-term results of selective dorsal rhizotomy for hereditary spastic paraparesis.

Pure hereditary spastic paraparesis usually presents with progressive weakness and spasticity of the legs, which is similar to spastic cerebral palsy. In this study selective dorsal rhizotomy (SDR) was performed to improve the spasticity of pure hereditary spastic paraparesis and the long-term results were followed. A series of four patients with pure hereditary spastic paraparesis diagnosed by a multidisciplinary team received SDR. The dorsal rootlets from the L2 to S1 levels were selectively resected under electrophysiological monitoring. The patients were followed up for more than 2 years to evaluate the outcome of surgery. There was a significant reduction in muscular spasm after SDR. Standing and walking stability were improved in all patients which led to improvement in walking posture and longer walking distance without assistance. No urinary retention, cerebrospinal fluid leak, surgical infection or kyphosis occurred. For severe pure hereditary spastic paraparesis, SDR can reduce muscle spasm and improve standing and walking stability. These results were stable throughout follow-up. SDR performed at the level of the conus medullaris through a laminectomy from T12 to L1 or L1 to L2 requires a shorter incision, laminectomy of fewer segments, and has a shorter operation time than the usual method (laminectomy from L2 to S1). Intraoperative electrophysiological monitoring is helpful to discriminate abnormal rootlets and protect sphincter function.

Delayed cerebrospinal fluid rhinorrhea after gamma knife surgery in a patient with a growth hormone-secreting adenoma.

We report a patient who developed delayed cerebrospinal fluid (CSF) rhinorrhea 11 years after gamma knife radiosurgery for a growth hormone (GH)-secreting adenoma. The treatment dose was 18 Gy for the tumor margin (50% isodose). One year later, an MRI of the head revealed that the tumor size had decreased. Eleven years later, the patient developed CSF rhinorrhea from the left nostril. Subsequent MRI examination revealed complete remission of the tumor in the sella turcica and the empty sella. The patient was admitted for direct endoscopic surgical repair of the skull base. We suggest that the cause of the CSF rhinorrhea is secondary empty sella. The other potential causes may be the original invasiveness of the tumor or delayed radiation damage to the mucous membranes of the skull. Long-term follow-up is required to monitor recurrence of CSF rhinorrhea.

Surgical treatment of patients with drug-resistant hypermotor seizures.

PURPOSE: To describe the clinical, electrophysiologic, neuroradiologic, and histologic findings in our patients with drug-resistant hypermotor seizures (HMSs) and to evaluate the outcome of their surgical treatment. METHODS: Twenty-three patients were identified by criteria for drug-resistant HMS. Surgical treatment and presurgical evaluation modalities including semiology, magnetic resonance imaging (MRI), interictal/ictal scalp video-EEG (electroencephalography), and intracranial recording were analyzed retrospectively. RESULTS: The common seizure frequency of 60-300 per month was observed among 15 patients. Sixteen patients (69.6%) experienced auras such as fear and palpitation. Marked agitation was observed in 12 patients and mild agitation in 11 patients. Groaning/shouting and asymmetric posturing were common accompanying symptoms. Asymmetric posturing was observed more often in patients with mild agitation than in those with marked agitation (p = 0.027). MRI detected focal abnormalities in six patients. Intracranial recording was conducted in 16 patients. The origins of seizures were localized in mesial frontal cortex in four patients, dorsolateral frontal cortex in four patients, and mesial temporal cortex in two patients. The epileptogenic zones (EZs) were resected from the frontal lobe in 21 patients and from the temporal lobe in 2 patients. The follow-up ranged from 12-60 months. Seventeen patients (73.9%) had been seizure-free, 11 of whom had presented with marked agitation (11 of 12) and 6 with mild agitation (6 of 11) (p = 0.069). Histologic examinations demonstrated focal cortical dysplasia (FCD) in 18 patients. DISCUSSION: The HMSs can originate from both the mesial and dorsolateral frontal cortex, and occasionally from the temporal lobe. Patients with drug-resistant HMSs should be recommended for resective surgical treatment.

Surgical treatment for musicogenic epilepsy.

We report a patient with medically intractable musicogenic epilepsy (ME) who was treated with surgery. Using the non-invasive methods of ictal and interictal electroencephalography (EEG), MRI, interictal single photon emission computed tomography and clinical manifestations, we first localized the musicogenic seizures (MS). The ictal onset zone was then further localized using intracranial EEG to the middle part of the left superior temporal gyrus. Surgical resection of the epileptogenic zone was then performed. The patient had two seizures within 2 weeks post-operatively, but has then had no seizures during the following year (Engel class II). The results suggest that patients who have medically intractable ME combined with unilateral ictal onset zones should be considered for the surgical treatment of epilepsy.

Surgery for perirolandic epilepsy: Epileptogenic cortex resection guided by chronic intracranial electroencephalography and electric cortical stimulation mapping.

SUBJECT: The objective of this study was to assess outcome with regard to seizure status and neurological function in patients undergoing resective surgery involving the perirolandic area. METHOD: All 15 patients who underwent perirolandic cortical resection between October 2006 and September 2007 at the Comprehensive Epilepsy Centre of Beijing Xuanwu Hospital were included in the study. The locations of functional cortical areas, ictal onset zones and epileptogenic lesions were mapped by chronic intracranial EEG recordings and electric cortical stimulation. Seizure outcome was determined using the modified classification of Engel and colleagues. Motor and sensory deficits were monitored. RESULTS: At last follow-up 5 patients (33%) were in Engel class I, 4 (27%) were in class II, 3 (20%) were in class III, and 3 (20%) were in class IV. Nine patients suffered immediate functional deficits; 8 of these recovered completely within 2 weeks to 3 months of surgery. One had mild persistent loss of finger motor control. CONCLUSION: After accurate presurgical evaluation using invasive recordings and functional brain mapping, epileptogenic cortical resection can give excellent results and few deficits in patients with perirolandic epilepsy.