Neuropsychiatric aspects of dementia with lewy bodies

Neuropsychiatric symptoms are prominent clinical features of dementia with Lewy bodies (DLB). Visual hallucinations have been reported to be particularly common. Auditory hallucinations, delusions, and depression also may be characteristic to DLB. Misidentification delusions may be more common than with other types of delusional syndromes. Supersensitivity to neuroleptic drugs is common, making treatment of these symptoms difficult, and newer, atypical compounds have been recommended. However, supersensitive reactions to these medicines have been reported. Patients with DLB, especially those with visual hallucinations, are reported to have a marked cholinergic deficit, and cholinergic drugs may be beneficial in reducing the neuropsychiatric symptoms.     

Hallucinations in schizophrenia

The prevalence of different types of hallucinations and their clinical correlates were examined in 117 DSM-III-R schizophrenic or schizoaffective disorder patients. Auditory hallucinations were by far the most common, followed by visual hallucinations, and then by tactile and olfactory or gustatory hallucinations. Auditory hallucinations were associated with an earlier age of first hospitalization among the schizophrenics. Global severity of the illness for schizophrenics was related to the presence of visual hallucinations, but not other types of hallucinations. Tactile and olfactory or gustatory hallucinations were strongly correlated with each other and with the severity of delusions for both schizophrenic and schizoaffective patients. The results suggest that important clinical differences exist between patients with different types of hallucinations, and that these clinical variables need to be controlled for in cross-cultural studies of hallucinations.     

Clinical presentation of neurocysticercosis-related epilepsy

Neurocysticercosis (NCC) is the most common parasitic infection of the central nervous system and a major risk factor for seizures and epilepsy. Seizure types in NCC vary largely across studies and seizure semiology is poorly understood. We discuss here the studies regarding seizure types and seizure semiology in NCC, and examine the clinical presentation in patients with NCC and drug-resistant epilepsy. We also provide evidence of the role of MRI and EEG in the diagnosis of NCC-related epilepsy. Focal seizures are reported in 60–90% of patients with NCC-related epilepsy, and around 90% of all seizures registered prospectively are focal not evolving to bilateral tonic–clonic seizures. A great number of cases suggest that seizure semiology is topographically related to NCC lesions. Patients with hippocampal sclerosis and NCC have different clinical and neurophysiological characteristics than those with hippocampal sclerosis alone. Different MRI protocols have allowed to better differentiate NCC from other etiologies. Lesions' stages might account on the chances of finding an interictal epileptiform discharge. Studies pursuing the seizure onset in patients with NCC are lacking and they are specially needed to determine both whether the reported events of individual cases are seizures, and whether they are related to the NCC lesion or lesions.This article is part of a Special Issue entitled “Neurocysticercosis and Epilepsy”.     

The semiology of febrile seizures: Focal features are frequent

ObjectiveTo clarify the semiology of febrile seizures (FS) and to determine the frequency of FS with symptoms suggestive of focal onset.MethodsFS symptoms in children were reported within 24 h of seizure onset by the parents using a structured questionnaire consisting principally of closed-ended questions. We focused on events at seizure commencement, including changes in behavior and facial expression, and ocular and oral symptoms. We also investigated the autonomic and motor symptoms developing during seizures. The presence or absence of focal and limbic features was determined for each patient. The associations of certain focal and limbic features with patient characteristics were assessed.ResultsInformation was obtained on FS in 106 children. Various events were recorded at seizure commencement. Behavioral changes were observed in 35 children, changes in facial expression in 53, ocular symptoms in 78, and oral symptoms in 90. In terms of events during seizures, autonomic symptoms were recognized in 78, and convulsive motor symptoms were recognized in 68 children. Focal features were evident in 81 children; 38 children had two or more such features. Limbic features were observed in 44 children, 9 of whom had two or more such features. There was no significant relationship between any patient characteristic and the numbers of focal or limbic features.SignificanceThe semiology of FS varied widely among children, and symptoms suggestive of focal onset were frequent. FS of focal onset may be more common than is generally thought.     

The treatment of recurring auditory hallucinations in schizophrenia with rTMS.

BACKGROUND: Auditory hallucinations are a common and disabling problem for many patients with schizophrenia and often fail to respond to optimal antipsychotic therapy. Repetitive transcranial magnetic stimulation (rTMS) has recently been trialled as an alternative treatment option for these patients. These studies have generally been positive, but treatment has only been provided for short periods of time and little is known about the longer-term impact of TMS on the course of hallucinations. METHOD: We describe two cases in which rTMS was provided to patients upon relapse of hallucinations following initial successful rTMS treatment in a clinical trial. RESULTS: A repeat course of rTMS resulted in a marked improvement in the symptoms experienced by these two patients. CONCLUSIONS: rTMS appears to have potential as a long-term treatment for patients with auditory hallucinations, but requires ongoing systematic investigation.     

Autosomal dominant lateral temporal epilepsy: two families with novel mutations in the LGI1 gene

PURPOSE: Mutations in the leucine rich, glioma inactivated gene (LGI1) were recently described in a small number of families with autosomal dominant lateral temporal epilepsy (ADLTE). ADLTE is characterized by partial seizures with symptoms suggestive of a lateral temporal onset, including frequent auditory aura. Here we report the results of clinical and genetic analyses of two newly identified families with ADTLE. METHODS: We identified two families whose seizure semiology was suggestive of ADLTE. Evaluation included a detailed history and neurologic examination, as well as collection of DNA. The coding sequence of the LGI1 gene from affected subjects from both families was analyzed for evidence of mutation. RESULTS: Each patient had a history of partial seizures, often with secondary generalization earlier in the course. Auditory aura was reported by approximately two thirds of affected patients in each pedigree. Novel mutations in LGI1 were detected in both families. A heterozygous single-nucleotide deletion at position 329 (del 329C) was detected in affected individuals from one family, whereas patients from the second family had a nonsynonymous variation, corresponding to C435G. CONCLUSIONS: We identified two novel mutations in the LGI1 gene. The phenotype of these two families was similar to that of other kindreds with ADLTE, as auditory aura was absent in one third of affected individuals. Our results further support that LGI1 mutations should be considered in patients with a history of partial seizures if the semiology of seizures is consistent with the onset in the lateral temporal lobe.     

Do recurrent seizure-related head injuries affect seizures in people with epilepsy?

Seizure-related head injuries (SRHIs) are among the most commonly encountered injuries in people with epilepsy (PWE). Whether head injury has an effect on preexisting epilepsy is not known. The purpose of this study was to systematically assess for any possible effects of SRHIs on seizure frequency and seizure semiology over a 2-year period. We identified 204 patients who have been followed at the Baylor Comprehensive Epilepsy Center from 2008 to 2010. SRHI occurred in 18.1% of the cohort. Most injuries (91%) were classified as mild. Though seizure frequency varied following head injury, overall seizure frequency was not significantly impacted by presence or absence of SRHI over the 2-year study period. Changes in seizure semiology were not observed in those with SRHIs. Although mild SRHI is common among PWE, it does not appear to have an effect on seizure characteristics over a relatively short period.     

Visual disturbances representing occipital lobe epilepsy in patients with cerebral calcifications and coeliac disease: a case series

Paroxysmal visual manifestations may represent epileptic seizures arising from the occipital lobe. In coeliac disease (CD) bilateral occipital calcifications and seizure semiology consistent with an occipital origin have been described, primarily in Mediterranean countries. By reporting three adult patients from an Australian outpatient clinic with visual disturbances, occipital cerebral calcifications, and CD, this study seeks to emphasise that CD should be considered even when patients of non-Mediterranean origin present with these symptoms. Seizure types included simple partial, complex-partial, and secondarily generalised seizures. The seizure semiology consisted of visual disturbances such as: blurred vision, loss of focus, seeing coloured dots, and brief stereotyped complex visual hallucinations like seeing unfamiliar faces or scenes. Symptoms of malabsorption were not always present. Neurological examination was unremarkable in two patients, impaired dexterity and mild hemiatrophy on the left was noted in one. Routine electroencephalography was unremarkable. In all cases, computed tomography demonstrated bilateral cortical calcification of the occipital-parietal regions. Magnetic resonance imaging showed no additional lesion. All patients had biopsy confirmed CD. Seizure control improved after treatment with gluten free diet and anticonvulsants. This report illustrates the association between seizures of occipital origin, cerebral calcifications, and CD even in patients not of Mediterranean origin.     

Age-dependent seizure semiology in temporal lobe epilepsy

OBJECTIVE: To examine the effects of age on different aspects of temporal lobe seizure semiology. METHODS: We performed a video analysis of 605 archived seizures from 155 consecutive patients (age 10 months to 49 years) selected by seizure freedom after temporal lobectomy. Eighty patients had hippocampal sclerosis (HS). Beside semiological seizure classification, we assessed age dependency of several axes of seizure semiology: (1) aura, (2) number of different lateralizing signs, occurrence of ictal (3) emotional signs, (4) autonomic symptoms, (5) automatisms, and (6) secondary generalization as well as (7) the ratio of motor seizure components. RESULTS: From the 155 patients, 117 reported aura, 39 had ictal emotional signs, 51 had autonomic symptoms, 130 presented automatisms, while 18 patients showed secondary generalization at least once during their seizures. Altogether 369 (median: 2/patient) different lateralizing signs were recorded. Frequency of HS (p < 0.001), ictal automatisms (p < 0.001), secondary generalization (p = 0.014), number of different lateralizing signs (p < 0.001) increased while the ratio of motor seizure component (p = 0.007) decreased by age. Auras, emotional symptoms, and autonomic signs occurred independently of patients' ages. Hippocampal sclerosis adjusted linear models revealed that the frequency of automatisms and secondarily generalized seizures as well as the number of different lateralizing signs are HS-independent significant variables. CONCLUSION: Our findings support that brain maturation significantly influences the evolution of some important aspects (motor seizures, lateralizing signs) of temporal lobe seizure semiology. Conversely, other aspects (aura, emotional, and autonomic signs) are independent of the maturation process. This is the first report investigating age dependency of epileptic seizure semiology comparing all age groups.     

Musical hallucinations and palinacousis

So far, little attention has been paid to the similarities between musical hallucinations and palinacousis. Since the authors found a 75-year-old woman suffering from both symptoms, the similarities were investigated. As a result, musical hallucinations have all the four components of palinacousis structurally, although there are some differences in content. Thus, there exist substantial similarities. Moreover, both symptoms are often associated with seizure activity and there have been several case reports where anticonvulsants were successfully used to treat both symptoms. These findings indicate the possibility that there may exist a common pathway generating musical hallucinations and palinacousis.     

Combining ictal surface-electroencephalography and seizure semiology improves patient lateralization in temporal lobe epilepsy

PURPOSE: The study goal was to assess the concordance of ictal surface-EEG and seizure semiology data in lateralizing intractable temporal lobe epilepsy (TLE) and to examine the benefits of the combined use of these two methods. METHODS: We independently analyzed the ictal recordings and clinical symptoms associated with 262 seizures recorded in 59 TLE patients. Each seizure was lateralized on the basis of (i) its associated ictal surface-EEG pattern according to a predefined lateralization protocol and (ii) its associated ictal and postictal seizure semiology according to strictly defined clinical criteria. Individual patients were also lateralized based on these data. RESULTS: Ictal surface-EEG findings lateralized 62.6% of seizures and 64.4% of patients. Seizure semiology findings lateralized 46.2% of seizures and 78.0% of patients. There was a high degree of concordance between lateralizations based on these two methods, for both individual seizures and individual patients. Combination of the information from the two methods allowed for lateralization in a greater proportion of both seizures (79.8%) and patients (94.9%). Combined EEG-seizure lateralization was concordant with the side of operation in 33 of 34 patients who underwent successful surgery (Engel's surgical outcome class I/II). CONCLUSIONS: In TLE, there is a high agreement between the lateralization of individual seizures and patients, which is based on ictal surface-EEG findings and seizure semiology. Furthermore, combination of these two methods improves the lateralization of individual seizures and patients. Thus, standardized combined EEG-seizure analysis is a valuable noninvasive tool in the presurgical evaluation of TLE.     

Culture and the prevalence of hallucinations in schizophrenia

Objective

Besides demographic, clinical, familial, and biographical factors, culture and ethnicity may plausibly influence the manifestation of hallucinations. The purpose of this study was to investigate the influence of culture on the frequency of different kinds of hallucinations in schizophrenia.

Method

Patients with a clinical diagnosis of schizophrenia were diagnosed by means of the Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition criteria. Seven independent samples were consecutively recruited in Austria, Lithuania, Poland, Georgia, Ghana, Nigeria, and Pakistan using identical inclusion/exclusion criteria and assessment procedures (N = 1080 patients total). The association of key demographic factors (sex and age), clinical factors (age at onset and duration of illness), and country of origin with hallucinations of different kinds was examined.

Results

The prevalence of various kinds of hallucinations was substantially different in the samples; however, the rank order of their occurrence was similar. Auditory hallucinations were relatively infrequent in Austria and Georgia and more prevalent in patients with an early age at onset of disease. Visual hallucinations were more frequently reported by the West African patients compared with subjects from the other 5 countries. Cenesthetic hallucinations were most prevalent in Ghana and in patients with a long duration of illness.

Conclusion

We hypothesize that the prevalence of the different kinds of hallucinations in schizophrenia is the result of the interaction of a variety of factors like cultural patterns as well as clinical parameters. According to our study, culture seems to play a decisive role and should be taken into account to a greater extent in considerations concerning the pathogenesis of psychotic symptoms.     

Psychiatric symptoms among patients with dementia seen in an ambulatory service

Subjects with dementia often display an array of neuropsychiatric symptoms that include disorders of mood, delusions, hallucinations, vegetative symptoms and psychomotor abnormalities. The present study was designed to investigate the prevalence of psychiatric morbidity amongst patients with the clinical diagnosis of dementia (ICD-10) assessed at a Memory Clinic in S?o Paulo-Brazil between February 1997 and May 1998. The mental and cognitive state of patients were assessed with an extended version of the SRQ-20 and the MMSE respectively. Thirty-four (45.3%) out of a total of 75 subjects scored 8 or more on the SRQ-20, indicating the presence of significant psychiatric morbidity. Depressive symptoms were reported by 69.3% of patients. Persecutory ideas and auditory hallucinations were observed in 20.0% and 16.0% of the sample respectively. Eight subjects (10.7%) described suicidal ideation--they all displayed depressive symptoms. Patients with scores on the SRQ-20 > or = 8 or who described suicidal ideation were significantly younger than their counterparts. Auditory hallucinations were more frequent amongst subjects with lower MMSE scores. There were no sex differences in the distribution of the psychiatric symptoms under investigation. The assessment of patients with dementia should always include a detailed psychiatric examination, as the detection and treatment of such symptoms may contribute to decrease the stress of patients and the burden on carers.     

Cortical activity associated with auditory hallucinations

Auditory hallucinations are one the most enigmatic and hampering symptoms associated with schizophrenia. Non-invasive functional imaging techniques have begun to delineate the underlying neuronal basis. We investigated the spontaneous magnetoencephalographic activity in a 33-year-old male schizophrenic patient and compared the results to those obtained from 13 healthy controls. Despite current neuroleptic medication (clozapine) the patient was still suffering from auditory hallucinations. Using the dipole density method, we were able to demonstrate an increase of fast MEG activity (12.5-30 Hz) in the left auditory cortex associated with hallucinations. This activity was absent in healthy controls. We conclude that an increase in fast MEG activity in the auditory cortex is a neurophysiologic correlate for auditory hallucinations in schizophrenia.     

Epilepsy Characteristics and Clinical Outcome in Patients With Mitochondrial Encephalomyopathy,Lactic Acidosis,and Stroke-Like Episodes (MELAS)

BackgroundEpileptic seizures in patients with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) are heterogeneous with no pathognomonic features. We reviewed epilepsy characteristics and clinical outcome exclusively in a pediatric population.MethodsTwenty-two children and adolescents (13 males) with confirmed mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes due to mitochondrial DNA A3243G mutation and epilepsy were recruited. Clinical data including seizure semiology, treatment response, neuroimaging findings, and electroencephalography were analyzed. We also examined the effect of the age at seizure onset and initial symptoms on the clinical variables.ResultsSeizure semiology and electroencephalography abnormalities showed no syndrome-specific findings. Focal seizures occurred in 21 of 22 subjects (95.5%), whereas generalized seizures developed in seven of 22 subjects (31.8%). Twenty of 22 subjects (90.9%) achieved partial to complete reduction of clinical seizures for more than one year with a combination of more than two antiepileptic drugs. The subgroup with earlier seizure onset presented significantly earlier and showed significantly higher rates of drug-resistant epilepsy compared with the late onset group, although there were no significant differences in the initial symptoms. The subjects with severe epileptic conditions tended to have more severe clinical dysfunction and more severe organ involvement.ConclusionsBoth focal and generalized seizures occurred in patients with MELAS. Epilepsy in this population is drug resistant, but a certain degree of clinical seizure reduction was achievable with antiepileptic drugs, with more favorable outcomes than historically expected. Close observation and active epilepsy treatment of individuals with MELAS episodes and earlier seizure onset might improve the prognosis.     

Behavioral abnormalities and psychiatric symptoms in Alzheimer's disease: preliminary findings

Behavioral abnormalities and psychiatric symptoms were assessed in 178 patients diagnosed as having Alzheimer's disease by NINCDS/ADRDA criteria. The subjects were selected from a defined catchment area and therefore were representative of a group of patients with Alzheimer's disease of varying severity. Auditory hallucinations were found in 10%, visual hallucinations in 13%, and delusions in 16%. Symptoms suggestive of depression were reported by 39% of the patients and features of depression observed in 25%. Twenty percent were aggressive, and 7% were sexually disinhibited. Nineteen percent exhibited excessive walking behavior and 10% binge eating. Nearly 50% of the sample were incontinent. Patients in the hospital were more often aggressive, incontinent, and seemingly less depressed. Patients with severe dementia displayed excessive walking behavior, were more likely to be incontinent, and reported less depressive symptoms than those with moderate or mild dementia.     

Seizure semiology and electroencephalography in young children with lesional temporal lobe epilepsy

PurposeThis study aimed to discuss the clinical features of seizure semiology and electroencephalography (EEG) in young children with lesional temporal lobe epilepsy (TLE).MethodChildren with lesional TLE received presurgical evaluation for intractable epilepsy. They were followed up for more than one year after temporal lobectomy. We reviewed the medical history and video-EEG monitoring of children with TLE to analyze the semiology of seizures and EEG findings and compared the semiology of seizures and EEG findings of childhood TLE and adult TLE.ResultsA total of 84 seizures were analyzed in 11 children (aged 23–108 months). The age of seizure onset was from 1 month to 26 months (a mean of 17.6 months). All of the patients exhibited prominent motor manifestations including epileptic spasm, tonic seizure, and unilateral clonic seizure. Seven children manifested behavioral arrest similar to an automotor seizure in adult TLE but with a shorter duration and higher frequency. The automatisms were typically orofacial, whereas manual automatisms were rarely observed. The EEG recordings revealed that diffuse discharge patterns were more common in younger children, whereas focal or unilateral patterns were more typical in older children. All of the patients were seizure-free after temporal lobectomy with more than one-year follow-up. All of the children had a mental development delay or regression; however, there was improvement after surgery, especially in those with surgery performed early.ConclusionIn contrast to TLE in adults, young children with lesional TLE probably represent a distinct nosological and probably less homogeneous syndrome. Although they had generalized clinical and electrographic features, resective epilepsy surgery should be considered as early as possible to obtain seizure control and improvement in mental development.     

Somatosensory auras in focal epilepsy: a clinical, video EEG and MRI study.

PURPOSE: To determine the clinical characteristics of somatosensory auras (SSA) and analyse features of seizure semiology predictive for localization in focal neocortical and limbic epilepsy. METHODS: This study analyses the clinical, video-EEG and MRI imaging features of 75 consecutive patients with focal epilepsy who described somatosensory auras at seizure onset to determine the frequency and localising value of SSAs in different types of focal epilepsy. Sensory characteristics, somatotopic distribution, evolution of the auras and subsequent ictal features in relation to MRI and EEG findings were analysed. RESULTS: The incidence of SSAs in 600 patients with focal epilepsy was 12%. Seventy-five patients were studied further: 77% reported tingling. Pain, thermal changes and a sense of movement or pulling were also reported. Distal unilateral auras in the hand and arm (46%) were most frequent and associated with a contralateral centroparietal focus. Contralateral auras were reported in 62% of lesional cases, focal cortical dysplasia was the commonest pathology in operated cases. Bilateral auras were associated with more diffuse pathologies or parasagittal foci. Evolution was centrifugal, somatotopic and usually unilaterally confined. Subsequent motor semiology was postural tonic, unilateral clonic, psychomotor or secondary generalized. CONCLUSION: SSA are highly correlated with centroparietal epilepsy but may occur in temporal lobe, mesial frontal and multifocal epilepsy. A lesional etiology including discrete dysplasias, tumours, ischemic and postencephalitic gliosis is likely.