An integrated neurocognitive and social-cognitive treatment for youth at clinical high risk for psychosis: Cognition for Learning and for Understanding Everyday Social Situations (CLUES).

BACKGROUND: Cognitive deficits, a core feature contributing to disability in schizophrenia, are present in milder form in individuals at clinical high risk (CHR) for psychosis. This study investigated the feasibility of Cognition for Learning and Understanding Everyday Social Situations (CLUES), an integrated neurocognitive and social cognitive treatment for youth at CHR. METHOD: This was an open, pilot feasibility trial. Seventeen individuals meeting CHR criteria were assessed prior to and following participation in CLUES for changes in symptoms, social and role functioning, and cognition. Participant attitudes towards CLUES were also examined. RESULTS: Participants significantly improved in social functioning [t(16) = -4.20, p = .001, d = 1.02], and trended for improvement in reaction time [t(15) = 2.09, p = .054, d = 0.52] from baseline to end of treatment. No other measures significantly changed. No participants transitioned to full psychosis during the treatment and follow up period. Participants reported they generally liked CLUES and found it helpful. CONCLUSION: While limited by the small sample size and the open label design, our preliminary results indicate that CLUES is feasible and shows promise in improving social functioning. However, further investigation is warranted in order to determine its efficacy. Future directions should include conducting a randomized controlled trial in order to compare the efficacy of CLUES to another intervention.

Reorganisation of cortical motor and language distribution in human brain.

BACKGROUND: The locations of cortex controlling motor, sensory, or language functions can change in adult humans under some circumstances, such as expanding tumours, trauma or continuous focal seizures. It is not clear what other circumstances might result in changes in cortical functional maps. METHODS: The results of extraoperative cortical mappings of motor, sensory, and language functions were compared in two epilepsy patients who underwent cortical resections on two separate occasions and who did not have brain tumours. RESULTS: It was found that the locations of motor functions could differ between the first and second procedures, but the locations of language functions were quite similar. The changes were not necessarily in or adjacent to epileptogenic regions or adjacent to resection boundaries. CONCLUSIONS: These findings support previous evidence indicating that cortical functional representations can change over time in humans, and suggest that these changes cannot be explained solely by lesion effects.

Clinical and EEG features of patients with EEG wicket rhythms misdiagnosed with epilepsy.

BACKGROUND: EEG wicket rhythms are 6- to 11-Hz medium-to-high voltage bursts that are sometimes misidentified as epileptogenic activity. The authors determined the clinical and EEG features of patients with wicket rhythms who had been incorrectly diagnosed with epilepsy. METHODS: Electroencephalographers at an epilepsy center re-read EEGs for patients referred for epilepsy management and identified patients with wicket rhythms. On further evaluation, the majority (54%; 25/46) of these patients were found not to have epilepsy. The authors compared the clinical and EEG features for the 25 patients with wickets and nonepileptic episodes with those of age- and sex-matched patients with partial-onset epilepsy using univariate and multivariate analysis. RESULTS: Several features distinguished patients with EEG wicket patterns and nonepileptic episodes (n = 25) from age- and sex-matched patients with epilepsy (n = 25): mid-adult age at onset of episodes (mean 38.4 years vs 19.8 years), prolonged clinical episodes (mean 155 minutes vs 2.3 minutes), and long duration of EEG wicket patterns (mean 0.66 seconds vs 0.11 second spikes). After controlling for other factors, patients without major confusion during episodes were unlikely to have epilepsy. CONCLUSION: Wicket patterns are often interpreted as epileptogenic. This distinctive EEG pattern should be considered in patients with clinical episodes atypical for epilepsy.

Electrocorticographic gamma activity during word production in spoken and sign language.

OBJECTIVE: To investigate the functional-neuroanatomic substrates of word production using signed versus spoken language. METHODS: The authors studied single-word processing with varying input and output modalities in a 38-year-old woman with normal hearing and speech who had become proficient in sign language 8 years before developing intractable epilepsy. Subdural electrocorticography (ECoG) was performed during picture naming and word reading (visual inputs) and word repetition (auditory inputs); these tasks were repeated with speech and with sign language responses. Cortical activation was indexed by event-related power augmentation in the 80- to 100-Hz gamma band, and was compared with general principles of functional anatomy and with subject-specific maps of the same or similar tasks using electrical cortical stimulation (ECS). RESULTS: Speech outputs activated tongue regions of the sensorimotor cortex, and sign outputs activated hand regions. In addition, signed word production activated parietal regions that were not activated by spoken word production. Posterior superior temporal gyrus was activated earliest and to the greatest extent during auditory word repetition, and the basal temporal-occipital cortex was activated similarly during naming and reading, reflecting the different modalities of input processing. With few exceptions, topographic patterns of ECoG gamma were consistent with ECS maps of the same or similar language tasks. CONCLUSIONS: Spoken and signed word production activated many of the same cortical regions, particularly those processing auditory and visual inputs; however, they activated different regions of sensorimotor cortex, and signing activated parietal cortex more than did speech. This study illustrates the utility of electrocorticographic gamma for studying the neuroanatomy and processing dynamics of human language.

The Heidenhain variant of Creutzfeldt-Jakob disease: clinical, pathologic, and neuroimaging findings.

We report two patients who developed isolated visual symptoms and signs as initial manifestations of Creutzfeldt-Jakob disease (CJD). Both patients had normal conventional T1- and T2-weighted brain magnetic resonance (MR) images; in one patient, early cortical abnormalities were detected by diffusion-weighted and fluid attenuated inversion recovery (FLAIR) magnetic resonance imaging (MRI). Results from the cerebrospinal fluid assay for the 14-3-3 brain protein were also negative in one patient, despite pathologic confirmation of CJD at autopsy. The Heidenhain variant of CJD should be considered in all patients who present with isolated visual manifestations, including homonymous hemianopsia and normal conventional brain MRI. Diffusion-weighted and FLAIR MRI may demonstrate early cortical abnormalities in patients with CJD. The CSF assay for the 14-3-3 protein may be normal, even in pathologically confirmed cases.

Anterior spinal cord injury with preserved neurogenic 'motor' evoked potentials.

OBJECTIVE: To describe two cases in which intraoperative monitoring of neurogenic 'motor' evoked potentials (NMEPs) did not identify a spinal cord injury that resulted in paraplegia. METHODS: Bilateral tibial nerve somatosensory evoked potential (SEP) and NMEP testing was performed in two patients during spinal deformity corrective surgery using standard stimulation and recording parameters. These potentials were obtained repetitively throughout the primary procedures and were performed again during a subsequent procedure that took place after the discovery of paraplegia. RESULTS: SEP and NMEP signals were preserved in both patients and no adverse events were identified during the initial procedures. Postoperatively, paraplegia was identified immediately upon recovery from anesthesia and preserved posterior column function was apparent on clinical exam. In the procedures following the discovery of paraplegia, SEP and NMEP signals remained comparable with signals elicited in the initial surgeries. CONCLUSIONS: Based on these cases and previously published experimental evidence, we conclude that while 'NMEPs' remain a useful second test of spinal cord function, they are not reliable indicators of motor tract function. An alternate term, such as 'spinally-elicited peripheral nerve responses' should be used.

Spontaneous regression of optic gliomas: thirteen cases documented by serial neuroimaging.

OBJECTIVE: To demonstrate spontaneous regression of large, clinically symptomatic optic pathway gliomas in patients with and without neurofibromatosis type 1 (NF-1). METHODS: Patient cases were collected through surveys at 2 consecutive annual meetings of the North American Neuro-Ophthalmology Society (NANOS) and through requests on the NANOSNET Internet listserv. Serial documentation of tumor signal and size, using magnetic resonance imaging in 11 patients and computed tomography in 2 patients, was used to evaluate clinically symptomatic optic pathway gliomas. All tumors met radiologic criteria for the diagnosis of glioma and 4 patients had biopsy confirmation of their tumors. In 3 patients, some attempt at therapy had been made many years before regression occurred. In one of these, radiation treatment had been given 19 years before tumor regression, while in another, chemotherapy had been administered 5 years before signal changes in the tumor. In the third patient, minimal surgical debulking was performed 1 year before the tumor began to shrink. RESULTS: Spontaneous tumor shrinkage was noted in 12 patients. Eight patients did not have NF-1. In an additional patient without NF-1, a signal change within the tumor without associated shrinkage was detected. Tumor regression was associated with improvement in visual function in 10 of 13 patients, stability of function in 1, and deterioration in 2. CONCLUSIONS: Large, clinically symptomatic optic gliomas may undergo spontaneous regression. Regression was seen in patients with and without NF-1. Regression may manifest either as an overall shrinkage in tumor size, or as a signal change on magnetic resonance imaging. A variable degree of improvement in visual function may accompany regression. The possibility of spontaneous regression of an optic glioma should be considered in the planning of treatment of patients with these tumors.

Managing aphasia: then and now.

The nature and attitudes of the professionals who 'manage' aphasia have changed significantly over the 75 years the IALP has existed. This review shows how IALP publications have reflected this, and considers recent developments, particularly in respect of the role played by the cognitive neuropsychological approach in evidence-based healthcare and by conversational approaches in the empowerment of aphasic people.

Brief bursts of pulse stimulation terminate afterdischarges caused by cortical stimulation.

OBJECTIVE: To determine whether cortical electrical stimulation can terminate bursts of epileptiform activity in humans, we used afterdischarges (ADs) as a model of epileptiform activity. METHODS: Cortical stimulation was performed for clinical localization purposes using subdural electrodes implanted in patients undergoing preresection evaluations for treatment of medically intractable seizures. We used 0.3-millisecond pulses of alternating polarity, repeated at 50 pulses/second. When stimulation produced AD, we often applied short additional brief bursts of pulse stimulation (BPS). We examined the effectiveness of BPS in aborting ADs in 17 patients using survival analysis. RESULTS: With BPS, ADs stopped within 2 seconds in 115 cases, 2 to 5 seconds in 22 cases, and in more than 5 seconds in 89 cases. Without BPS, ADs stopped within 2 seconds in 21 cases, 2 to 5 seconds in 114 cases, and in more than 5 seconds in 340 cases. BPS was an effective method to abort ADs (Cox proportional hazards model: p<0.0001). At any time during the course of ADs, the instantaneous rate of stopping ADs within 2 seconds after BPS was applied was 4.6 times greater than when BPS was not applied (95% CI = 3.7, 5.7). In eight cases, ADs progressed to the occurrence of clinical seizures, always when BPS was not applied. CONCLUSIONS: Afterdischarges significantly decreased in duration after we applied brief bursts of pulse stimulation. Although afterdischarges are not identical to spontaneous epileptiform activity, these results support the idea that electrical stimulation, applied in an appropriate manner at seizure onset, could abort seizures in humans.

Effect of functional endoscopic sinus surgery on bronchial asthma outcomes.

BACKGROUND: For more than 70 years, the coexistence of asthma and paranasal rhinosinusitis has been noted in the medical literature. Causal relationships have been proposed but not proved. To date, limited evidence exists suggesting that asthma improves after surgical correction of rhinosinusitis. OBJECTIVE: To determine whether asthma control improved after first-time functional endoscopic sinus surgery (FESS). PATIENTS AND METHODS: A retrospective medical record analysis was performed on 13 patients with chronic bronchial asthma who underwent FESS for medically refractory chronic rhinosinusitis. Patients received comprehensive asthma care before and after FESS (mean, 19.3 and 33.1 months, respectively). Outcomes analyzed included pre- and post-FESS individual and group mean asthma symptom scores, medication use scores, pulmonary function test results, and emergency department visits or hospital admissions for asthma. Patient medical records were obtained from a private allergy-immunology practice affiliated with a medical school. The surgical procedure was performed at a tertiary care teaching hospital by a single ear, nose, and throat surgeon (R.L.). RESULTS: Following FESS, there was no statistically significant change in group mean asthma symptom scores, asthma medication use scores, pulmonary function test results, and the number of emergency department visits or hospital admissions. Only a few patients demonstrated statistically significant improvement after FESS in asthma symptom scores (1 patient), medication use scores (1 patient), or pulmonary function test results (2 patients). CONCLUSIONS: The data do not support the hypothesis that first-time FESS for medically refractory chronic rhinosinusitis in adult patients with chronic asthma leads to reduced postoperative asthma symptoms or asthma medication use or improved pulmonary function. Based on this limited study, a reexamination of the benefits of sinus surgery to coexisting asthma is in order.

Infantile cerebral aneurysms with visual pathway compression.

Intracranial aneurysms are rare in infancy. The commonest presentation is intracranial hemorrhage, but signs of mass effect are more frequent than in adults. We report 2 infants with cerebral aneurysms, one presenting with macrocephaly and another with strabismus. Both had visual loss and optic disc pallor; MRI revealed a suprasellar mass and anterior visual pathway compression. In both cases, the preoperative diagnosis was craniopharyngioma. It is essential to recognize that, although exceedingly uncommon, cerebral aneurysms do occur in infants and have features that differ from those in adults.

An automated injection system (with patient selection) for SPECT imaging in seizure localization.

PURPOSE: Ictal single-photon-emission computed tomography (SPECT) provides lateralization but has technical limitations: (a) a "truly ictal" injection must be shortly after seizure onset; (b) therefore, a seizure of brief duration may be missed; (c) more than one patient may need testing at any given time; (d) a trained health professional must stay next to each patient to inject; and (e) because the radionuclide is placed in the syringe in advance of the injection, decay of the radioactive element could result in less than optimal uptake, if the same volume of material were to be used regardless of the time after ligand preparation. METHODS: We developed an automated method of ligand injection that shortens time and increases efficiency of ictal SPECT ligand injection. By using an experimental setup, we compared manual injection times with times using an automated injection system. We determined relative costs and efficiency in work hours for the manual and automated methods. RESULTS: Injection times were 8-14 s with automated versus 19-26 s with manual injection. Readjusting volume for "ligand" decay was simple and accurate with the automated system. Injection times for clinical SPECT studies in three patients were 13, 13, and 12 s, respectively. The price of one pump equals 120 work hours of a nurse or 24 ictal injection attempts. Much of the nurse's time is "wasted" because no seizure occurs. CONCLUSIONS: The method can be more efficient of staff, shorten injection time, and facilitate obtaining "truly ictal" injections. It allows more cost-effective use of personnel.

Painful stimuli evoke potentials recorded from the parasylvian cortex in humans.

Cutaneous stimulation of the face and hand with a CO2 laser in three awake patients evoked potentials (LEPs) recorded from the dominant left parasylvian cortex. These were recorded by means of a subdural grid of electrodes implanted for evaluation of epilepsy. Stimulation of the contralateral face resulted in waveforms consisting of a negative potential (N2, 162 +/- 5 ms; mean +/- SE) followed by a positive potential (P2, 340 +/- 18 ms). Both waves occurred at longer latency after hand than after facial stimulation. N2 and P2 potentials recorded from the grid correspond well in morphology to those recorded from the scalp in four additional patients tested with the same stimulation paradigm. The N2 waves recorded from the subdural grid occurred at significantly shorter latencies than did those recorded from the scalp (184 +/- 6 ms), but the P2 waves at the grid occurred at significantly longer latencies than did those recorded at the scalp (281 +/- 13 ms). The amplitudes of the potentials recorded from the grid were maximal over the parietal operculum both for contralateral stimulation of the face or hand and for ipsilateral stimulation of the face. Potentials also were recorded in this area after stimulation of the ipsilateral hand. The cortical distributions of these potentials suggest that their generators are located in the parietal operculum or in the insula, or in both, consistent with previous PET, magnetoencephalographic, and scalp LEP source analyses. These previous analyses provide indirect evidence of nociceptive input to parasylvian cortex because the interpretation of each analysis incorporates multiple assumptions. The present results are the first direct evidence of nociceptive input to the human parasylvian cortex.