Surgical resection for intractable epilepsy in "double cortex" syndrome yields inadequate results.

PURPOSE: To analyze the results of surgical treatment of intractable epilepsy in patients with subcortical band heterotopia, or double cortex syndrome, a diffuse neuronal migration disorder. METHODS: We studied eight patients (five women) with double cortex syndrome and intractable epilepsy. All had a comprehensive presurgical evaluation including prolonged video-EEG recordings and magnetic resonance imaging (MRI). RESULTS: All patients had partial seizures, with secondary generalization in six of them. Neurologic examination was normal in all. Three were of normal intelligence, and five were mildly retarded. Six patients underwent invasive EEG recordings, three of them with subdural grids and three with stereotactic implanted depth electrodes (SEEG). Although EEG recordings showed multilobar epileptic abnormalities in most patients, regional or focal seizure onset was recorded in all. MRI showed bilateral subcortical band heterotopia, asymmetric in thickness in three. An additional area of cortical thickening in the left frontal lobe was found in one patient. Surgical procedures included multiple subpial transections in two patients, frontal lesionectomy in one, temporal lobectomy with amygdalohippocampectomy in five, and an additional anterior callosotomy in one. Five patients had no significant improvement, two had some improvement, and one was greatly improved. CONCLUSION: Our results do not support focal surgical removal of epileptogenic tissue in patients with double cortex syndrome, even in the presence of a relatively localized epileptogenic area.

Landau-Kleffner syndrome. Treatment with subpial intracortical transection.

Landau-Kleffner syndrome (LKS) is an acquired epileptic aphasia occurring in childhood and associated with a generally poor prognosis for recovery of speech. It is thought to be the result of an epileptogenic lesion arising in speech cortex during a critical period of development. Utilizing a new surgical technique designed to eliminate the capacity of cortical tissue to generate seizures while preserving the normal cortical physiological function, we have treated 14 children with aphasia, seizures and a severely abnormal EEG by multiple subpial transection of the epileptogenic cortex. Seven of the 14 patients (50%) have recovered age-appropriate speech, are in regular classes in school and no longer require speech therapy. Four of the 14 (29%) have shown marked improvement, are speaking and understanding verbal instruction but are still receiving speech therapy. Thus, 11 of the 14 (79%), none of whom had used language to communicate for at least 2 years, are now speaking--a rate of sustained improvement considered unusual in this disorder. This study documents the value of a treatment modality not previously used in LKS. Success depends on selection of cases having severe epileptogenic abnormality that can be demonstrated to be unilateral in origin despite a bilateral electrographic manifestation.

Intramedullary abscess: a report of two cases and a review of the literature.

Two cases of chronic spinal cord abscess are reported, and the relevant literature is reviewed with emphasis on the last 20 cases. Presentation, cause, modern diagnostic testing, operative findings, treatment choices, and prognosis are all discussed. Significant changes in the presentation, management, and outcome in these more recent cases are emphasized.

Recurrent intracranial epithelioid hemangioendothelioma associated with multicentric disease of liver and heart: case report.

Epithelioid hemangioendothelioma is an unusual vascular neoplasm with prominent cytoplasmic vacuolization representing primitive lumen formation. A case is presented of this unique vascular neoplasm in a woman with a seizure disorder who had cardiac, hepatic, and recurrent nervous system lesions. To our knowledge, this is the third known case of intracranial epithelioid hemangioendothelioma. Emphasis is placed on the indolent course of this rare neoplasm, with a recommendation for aggressive surgical treatment and diligent follow-up.

Magnetic resonance imaging as a sensitive and specific predictor of neoplasms removed for intractable epilepsy.

Twenty-three patients had magnetic resonance imaging (MRI) and computed tomography (CT) of the head prior to surgery for medically intractable epilepsy. Eleven patients had neoplasms, mostly astrocytomas. Six of the 11 tumors were seen on CT. In five of the six cases, the MRI showed a focal area of increased signal on T2-weighted images. All 11 tumors were detected by MRI. None of the non-neoplastic lesions produced an abnormal T2-weighted signal area on MRI. Only one of the non-neoplastic lesions was seen on both CT and on MRI. MRI allowed clear discrimination between tumors and non-neoplastic lesions in patients coming to surgery for intractable epilepsy.

Multiple subpial transection: a new approach to the surgical treatment of focal epilepsy.

A new operative approach has been designed for the relief of medically intractable focal epilepsy. It is intended particularly to be used in those cases where the epileptogenic lesion lies in "unresectable" cortex; that is, those cerebral regions subserving speech, memory, and primary motor and sensory function. The procedure is based upon experimental evidence indicating 1) that epileptogenic discharge requires substantial side-to-side or horizontal interaction of cortical neurons, and 2) that the major functional properties of cortical tissue depend upon the vertical fiber connections of the columnar units. The technique requires severing of tangential intracortical fibers while preserving the vertical fiber connections of both incoming and outgoing nerve pathways and of the penetrating blood vessels which also have a vertical orientation. In this study, the effect of multiple subpial transection was assessed on both function and seizure control. The effect on function was reviewed in 32 cases; only 20 cases were evaluated with respect to seizure control, since a follow-up period of 5 years or more (5 to 22 years) is required before conclusions can be drawn. Multiple subpial transection was applied to the precentral gyrus in 16 cases, the postcentral gyrus in six, Broca's area in five, and Wernicke's area in five. With respect to function, the major finding was that none of the 32 patients has suffered a clinically significant behavioral deficit (although subtle deficits could be detected by careful neurological examination). Complete control of seizures has been obtained in 11 (55%) of the 20 cases evaluated. Nine patients developed recurrent seizures consequent to progressive disease unsuspected before operation (Rasmussen's encephalitis in five, tumor in three, and subacute sclerosing panencephalitis in one). In none of these cases, however, did the recurrent seizures arise in the transected zone. Thus, the results indicate that multiple subpial transection is about as effective as standard excisional therapy, and can be successfully employed when epileptogenic lesions encroach upon cortical territories, the removal of which would be functionally incapacitating.

Prevalence of psychologic disorders after surgical treatment of seizures.

To investigate whether surgical treatment of refractory epilepsy is associated with increased risk for serious psychopathology, 25 treated patients were compared with 25 current candidates for surgery matched on demographic and neuroepileptic characteristics. Diagnoses were made by the National Institute of Mental Health Diagnostic Interview Schedule. No differences between groups in lifetime or point prevalence rates were significant. The rate of psychosis in the postoperative group (8%) approximated the lower estimates in previous studies. Thus, surgical treatment of seizures did not increase the risk for psychopathology. However, patients with temporal lobe electroencephalogram foci or tumor as the epileptogenic lesion were more likely to have serious disorders than other patients. Also, anxiety disorders were more prevalent in our patient groups than in the general population.

Infarction after surgery for focal epilepsy: manipulation hemiplegia revisited.

The success of surgery for seizure focus resection depends on postoperative reduction or disappearance in number of seizures, improvement in psychosocial functioning, and low morbidity and mortality. Permanent neurologic sequelae are most often not discussed in this context. Deficits more than a superior quadrantanopsia are not expected after temporal lobectomy. Four cases of ischemic stroke after seizure focus resection, each distant from the site of tissue removal, are reported. These are the first such radiologically documented reports of "manipulation hemiplegia." The permanent neurologic deficits are not attributed to resected tissue or edema.

Chemonucleation and changes observed on lumbar MR scan: preliminary report.

This study examines the relation between the postchemonucleation clinical outcome and changes observed on the lumbar MR scan. Eight of 12 chemonucleated disks showed clinical improvement at the last follow-up, while the other four showed no improvement. In the cases that showed improvement there was a decrease in one or both dimensions of the defect in the thecal sac. Seven of eight showed an increase in the peridisk signal. Where there was no clinical improvement there were fewer decreases in the size of the defect, and three of four showed no increase in the peridisk signal.

Hemorrhagic complications after the lumbar injection of chymopapain.

There are few reports of hemorrhagic central nervous system complications after chymopapain injection in humans. Two patients are reported who developed hemorrhagic complications after the lumbar injection of chymopapain. The first developed a hemorrhagic encephalomyelopathy followed by clinically suspected acute arachnoiditis, which responded to high doses of dexamethasone. The second patient developed subarachnoid hemorrhage secondary to vertebral artery aneurysm rupture after the injection of chymopapain.

Safety and efficacy of chymopapain (Chymodiactin) in herniated nucleus pulposus with sciatica. Results of a randomized, double-blind study.

A double-blind, randomized trial was conducted to compare the efficacy of intradiskal injection of chymopapain (Chymodiactin) with injection of placebo in patients with a herniated lumbar disk. Patients were randomly assigned to either placebo or drug regimens and followed up for six months. The primary measure of performance was agreement by patient and surgeon that further intervention was not necessary. At any time that patient and investigator agreed that treatment had failed, the patient was classified as a failure and the treatment code was broken. Of 53 placebo-treated patients, 31 failed by this criterion. Of 55 drug-treated patients, 15 failed. Placebo-treated patients who failed were allowed to receive drug treatment, and 29 (91%) of 32 were treated successfully. This study demonstrates that chymopapain is more effective than placebo for treatment of patients with a herniated lumbar disk.

Anaphylaxis secondary to chymopapain.

Anaphylaxis occurs in approximately 1% of the patients treated with chymopapain. The incidence of anaphylaxis is ten times higher in women than in men. The severity of the reaction may be attenuated by pretreatment with H1- and H2-histamine receptor antagonist. Once anaphylaxis occurs, prompt and correct treatment must be instituted.

Methohexital anesthesia in the surgical treatment of uncontrollable epilepsy.

Twenty-five patients (aged 3 to 39 years) were anesthetized with methohexital for electrocorticographic mapping and resection of epileptogenic foci. These patients have been compared with 11 patients (aged 11 to 40 years) who had the same surgical procedure performed while they were awake because their epileptogenic foci were near the speech or motor areas. All patients received morphine and droperidol to produce analgesia and sedation, and a field block was established with local anesthetics. In the 25 patients, general anesthesia was induced with methohexital, 1.5 mg/kg. and maintained with a 0.1% infusion. After intubation, ventilation to a PaCO2 of 30 mm Hg was maintained with O2/air. A resectable abnormal electroencephalogram focus was localized in every case. All but two of the patients awoke promptly in the operating room, allowing extubation and participation in neurologic assessment. None remembered the procedure. The incidence of improvement of seizures in patients given methohexital was similar to that in patients who had surgery while awake. Unlike many general anesthetics that depress epileptogenic activity, methohexital activates seizure activity and can therefore be used for the dual purpose of producing general anesthesia and enhancing electrocorticographic delineation of epileptogenic foci.

Organic status, psychological disturbance, and pain report characteristics in low-back-pain patients on compensation.

The relationship betewwn compensation and three variables--psychologic disturbance, organic status, and pain report characteristics--was assessed. Patients on compensation were clinically similar to patients not on compensation in the relative frequency of cases of psychologic disturbance and nonorganic findings in each group. Patients on compensation differed only when objective evidence of organic disease and psychologic stability was present. Under these circumstances, the compensation group used 43% more words to describe their pain and endorsed more pain qualities on five independent dimensions of pain. These results indicate that compensation primarily affects the description of low-back pain in cases where objective evidence of injury is present and leads to an intensification of sensory discomfort. Little justification was found for the atmosphere of suspicion that surrounds patients on compensation who have no evidence of organic disease.

A comparison of patients treated by chymopapain and laminectomy for low back pain using a multidimensional pain scale.

Seventy-seven patients treated by chymopapain and laminectomy were compared before, and 6 and 14 weeks after, treatment. A standardized, multidimensional scale of low back pain providing scores on 7 independent dimensions of sensory and affective discomfort and one measure of intensity were used. Fourteen weeks after surgery, patients treated by chymopapain were functioning as well as those treated by laminectomy. Major differences, however, characterized the course of recovery. Chymopapain produced rapid change, which was maintained over the 3 1/2-month period. Healing following laminectomy was slower, but the end results were essentially the same. These findings, using sophisticated measurement techniques, support previous research suggesting that chymopapain is an effective alternative to laminectomy in the treatment of lumbar disk disease.