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This blockbuster issue of Neuropsychoanalysis is packed with examples of the theoretical generativity of neuropsychoanalysis, which may have far-reaching clinical consequences. A series of pieces i...

The paper describes a patient with psychotic depression following left inferior parietal stroke. He had a dominant delusion: a belief that he was buried alive and had died thereafter. This ...

Objectives The aim of this study was to examine awareness of decline in memory and in language in individuals with Alzheimer's disease (AD), by comparing participant and informant ratings, as well as these ratings and actual test performance. Methods We analyzed data from 149 individuals with AD enrolled in the Alzheimer's Disease Neuroimaging Initiative (ADNI) who filled the Everyday Cognition questionnaire and performed memory and language tasks. Results Participants provided significantly lower assessments of decline than did informants for both memory and language. There was a negative association between informant ratings and memory test scores but no association between participant ratings and memory test scores. Both participant and informant ratings correlated negatively with performance on the language tests. Informant, but not participant, ratings contributed to the prediction of one memory variable beyond demographic factors. Participant ratings contributed to the prediction of language scores beyond demographic factors more than did informant ratings. Conclusions The findings reflect better awareness of decline in language than of decline in memory in individuals with AD.

Studies indicate that individuals with borderline personality disorder (BPD) often demonstrate attenuated pain perception, termed hypoalgesia. This study examines the hypothesis that body awareness moderates the association between BPD and pain perception. Participants consisted of 46 women diagnosed with BPD and 47 healthy controls. Sensory testing included the measurement of heat-pain thresholds, ratings of suprathreshold stimuli, measurement of temperature evoking moderate pain, and temporal summation of noxious mechanical stimuli. Body awareness was assessed by a self-report questionnaire. As hypothesized, among subjects with low levels of body awareness, those with BPD demonstrated hypoalgesia as manifested in their lower suprathreshold pain ratings and moderate pain evoked by higher temperature, in comparison with the controls. Among those with high levels of body awareness, BPD subjects demonstrated increased reactivity to pain as manifested in their higher pain ratings and moderate pain evoked by lower temperature, in comparison with the controls.

The three Original Articles in the current issue represent widely divergent theoretical explorations. At the same time, they all concern themselves with the very nature of the disciplines of neurop...

Background Conversion disorder (CD) is a largely enigmatic disorder, one that requires a thorough ruling-out process. Prior research suggests that metaphors and conceptualization are rooted in physical experience, and that we interpret our affective world through metaphors. Spatial metaphors (interaction of affect and vertical space) are a prominent example of the grounding of metaphors. This is a relatively unpaved direction of research of CD. Objectives The present pilot study sought to explore this view by investigating the “healthy is up, sick is down” spatial metaphors (e.g., “fell ill” and “top shape”) in patients with CD, examining the correlation between the processing of bodily-related words, CD, and vertical space. We hypothesized that patients with CD, who experience their bodies as ill, will demonstrate a downwards bias when processing bodily-related words; corresponding to the “healthy is up, sick is down” spatial metaphor. Methods A total of 8 female patients (ages M-38.13 SD-10.44) and 42 female controls (ages M-36.4 SD-14.57) performed a visual attention task. Participants were asked to identify a spatial probe at the top or the bottom of a screen, following either a bodily related (e.g., arm) or non–bodily related (e.g., clock) prime word. Results As predicted, when processing bodily-related words, patients with CD demonstrated a downwards attention bias. Moreover, the higher the patient's level of somatization, the faster the patient detected lower (vs upper) spatial targets. Conclusions This study suggests that the changed health paradigm of patients with CD is grounded in sensorimotor perception. Further research could propose new diagnostic and treatment options for CD.

Objectives The aim of this study was to examine whether the discrepancy between participant and informant estimation of memory decline can predict MCI prognosis. Methods Analyses involved data from individuals with MCI enrolled in the Alzheimer's Disease Neuroimaging Initiative (ADNI) who filled the Everyday Cognition questionnaire. Participants who underestimated (N = 112) and overestimated (N = 157) their memory decline were compared on memory tasks, brain volume, and cerebrospinal markers, at study entry and after 24 months. Results Individuals who underestimated their memory decline performed more poorly on memory tests, had smaller hippocampus volume, and greater Alzheimer's disease pathology than did individuals who overestimated their cognitive decline. Longitudinal comparisons demonstrated that individuals who underestimated their decline deteriorated more significantly in memory and in brain measures. Conclusions Underestimation of memory decline should raise clinicians' suspicion of the existence of AD pathology in individuals with MCI.

BACKGROUND Ample evidence suggests that individuals with borderline personality disorder (BPD) exhibit hyposensitivity to pain. Since the underlying mechanism of the pain hyposensitivity is unknown, we tested here for the first time whether this hyposensitivity is pain specific or exists also for innocuous sensation, and whether it is associated with enhanced descending pain modulation capabilities. METHODS Participants were 55 women; 22 patients with BPD and 33 matched healthy controls. Testing included the measurement of warmth sensation threshold (WST), heat-pain threshold (HPT), pain adaptation, conditioned pain modulation (CPM) and temporal summation of heat-pain (TSP). The level of dissociation was also evaluated. RESULTS Women with BPD had higher WST and HPT compared with healthy controls. Moreover, women with BPD had greater magnitude of pain adaptation and CPM as well as higher dissociation level compared to controls. In neither the BPD nor the control group did WST and HPT correlate with pain adaptation, CPM or dissociation. In the BPD group only, HPT inversely correlated with the magnitude of TSP. CONCLUSIONS Women with BPD present generalized hyposensitivity to both innocuous and noxious stimuli. Furthermore, women with BPD exhibit more efficient pain inhibition capabilities than healthy controls. While efficient pain modulation may underlie pain hyposensitivity in BPD, both traits may exist independently from each other, or may be moderated by another factor such as dissociation. SIGNIFICANCE On the basis of testing pronociceptive and antinociceptive components among individuals with BPD and healthy controls, this study reveals enhanced ability to inhibit pain among woman with borderline personality disorder (BPD) which may underlie hyposensitivity to both noxious and innocuous stimuli and perhaps also self-injurious behaviour among these individuals. The study contributes novel information on possible mechanisms involved in BPD manifestations.

Individuals with semantic dementia (SD) show progressive worsening of lexical-conceptual single word knowledge alongside preservation of nonsemantic aspects of language. The current study examines morphological processing in SD. S.H.S., a Hebrew-speaking person with SD, completed eight tasks that tested inflection, linear derivation, and nonlinear derivation at three time points over six and a half years. S.H.S. correctly produced plural nouns and judged the grammaticality of noun-verb and noun-adjective inflectional agreement. A steeper decline was seen in the ability to judge irregularly versus regularly inflected forms. S.H.S. judged nationality suffixes accurately and produced diminutive and agentive suffixes successfully. He also relied on morphological decomposition when performing lexical decision. Judgement of sentences consisting of derived verbal participles was intact with regard to morphological considerations alone, but impaired when semantic considerations determined grammaticality. S.H.S.'s performance suggests that structural aspects of word knowledge might be functional in the face of a severe semantic impairment.

Objective To assess psychological factors of individuals suffering from conversion disorder/Functional Neurological Disorder (CD/FND) as observed through their motor behavior. Methods We analyzed the psychomotor behavior of 6 patients (all male, average age – 52.8 years) with CD/FND in a specialized clinic using a binary motor - psychological developmental paradigm (Emotorics - Emotive Body Movement Mind Paradigm [Emotorics-EBMMP]). Results All patients showed dominance of an early developmental prototype (P0) characterized by flexed postures resembling the infant held by his caregivers and relative paucity of a more developed prototype (P1) characterized by erect position typical for a face to face interaction. Conclusions The P0 preponderance suggests impingement on the core self and regression to/or fixation in an early developmental stage.

Charcot-Marie-Tooth type 1A (CMT1A) is an autosomal dominant polyneuropathy due to a 1.5 Mb tandem duplication in chromosome 17p11.2, containing the PMP22 gene. This mutation is not modified during inheritance. We set forth to test the hypothesis that in a subgroup of CMT1A patients there is clinical anticipation, namely an increase in disease severity over generations. Thirty-nine CMT1A mutation-positive patients in 16 families and 23 parent-offspring pairs were evaluated. This included 14 families with 2 generations and 2 families with 3 generations. Age of presentation was assessed by interviewing the patients and clinical severity was measured using the CMT neuropathy score (CMTNS). In 21/23 parent-child pairs and 14/16 families, there was an earlier age of presentation in children of genetically affected parents. The mean age of onset in the progeny was 12.61 years compared to 41.22 years in the parent generation, (p < 0.001).Mean severity in the younger generation was slightly higher than that of the parent generation. When corrected for the age difference, the trend for a worse phenotype in the younger generation became statistically significant (p < 0.02,Wilcoxon signed rank test). Our findings suggest that in a subgroup of CMT1A patients there is an increase in clinical severity over generations. The mechanism responsible for this observation remains unknown. Our findings should be validated on a larger cohort of CMT1A families.

The alien hand syndrome is a deeply puzzling phenomenon in which brain-damaged patients experience their limb performing seemingly purposeful acts without their intention. Furthermore, the limb may interfere with the actions of their normal limb. We report a case of alien hand syndrome following a left medial frontal and corpus callosum ischemic lesion. From our clinical observations and the patient's performances on experimental tasks, we postulate that three factors contribute to the sense of alienness: First, the errant limb must be disinhibited and disproportionately reactive to external environmental stimuli. Second, the limb is under less volitional control and produces perseverative movements in which motor stereotypies are concatenated. Consequently, the disinhibited limb perseverates on external stimuli and appears purposeful, despite not being engaged in true goal-directed intentions. Finally, the patient needs to have a relatively intact action-monitoring system to be aware of the abnormal movements as they are occurring.

Patients with alien hand (AH) syndrome from medial frontal lesions exhibit involuntary but seemingly purposeful contralesional upper limb movements. Two observations about AH patients have received little, if any, experimental confirmation. The first is that AH is triggered opportunistically by nearby objects. The second is that AH behaviors are increased in conditions of fatigue or anxiety, i.e. under reduced attentional control. A prominent account explains AH as reduced intention-driven (endogenous) executive control. This account predicts that erroneous AH behaviors should be driven by environmental (i.e. exogenous) factors, such as distractor proximity to the hand. AH errors should be less influenced by the intention or action plan (i.e. endogenous factors), such as the semantic relatedness of distractors to targets. Moreover, due to capacity limitations of the endogenous controller, AH behaviors should increase under conditions of secondary task load. We tested these predictions with an AH patient in two experiments using a naturalistic coffee-making task. Experiment 1 demonstrated that the affected hand was highly perseverative and strongly influenced by exogenous but not endogenous factors. The non-alien hand made fewer errors. Experiment 2 showed that there was a disproportionate increase in perseverations and exogenous errors of the affected hand under secondary task load. The non-alien hand was significantly less disrupted by dual task conditions. These data provide experimental support for previous anecdotal observations about AH behavior in naturalistic settings, and are consistent with a unilateral defect in endogenous control.

Background: Frontotemporal dementia with parkinsonism linked to chromosome 17q21–22 (FTDP-17) is an autosomal dominant tauopathy manifested by a variable combination of personality changes, cognitive decline and hypokinetic-rigid movement disorder. Significant clinical and pathological heterogeneity of FTDP-17 is related in part to more than 20 different pathogenic mutations identified in the tau gene. Among others, the P301S mutation has been previously reported in three families of European and one of Japanese origin presenting with different clinical phenotypes. Objectives To report a three-generation family of Jewish-Algerian origin with FTDP-17 due to the P301S tau mutation. Methods: Clinical, neuropsychological and neuroimaging evaluation of 3 patients, tau genotyping, and pathological study of the proband. Results: The 3 affected family members had a fairly stereotyped clinical course with early personality changes from their late 30s followed within a period of 1–2 years by a progressive cognitive and motor deterioration eventually leading to a state of akinetic mutism or death 3–5 years after the initial symptoms. The main clinical manifestations included severe dementia and hypokinetic-rigid movement disorder associated with supranuclear gaze impairment, pyramidal signs and frontal release signs. Brain imaging disclosed a variable degree of frontotemporal atrophy, ventriculomegaly and regional cerebral hypoperfusion or glucose hypometabolism. Frontal lobe biopsy in the proband revealed weak tau immunoreactivity in a few cortical neurons, in rare neurites and in some glial cells with no neurofibrillary tangles. Molecular DNA analysis identified a P301S mutation in exon 10 of the tau gene. Conclusions: The observed clinical features further expand the reported P301S phenotype and confirm a more aggressive course of the disease than in the other known tau mutations.

Objectives Hemoptysis, an important and alarming symptom, often indicates serious disease. This study was designed to assess the different causes of hemoptysis, the relative importance of the different diagnostic modalities employed, and the outcome in an Israeli population cohort. Design A retrospective analysis of 208 patients with hemoptysis at the Hadassah University Hospital, Jerusalem, Israel between January 1980 and August 1995. Results Bronchiectasis (20%), lung cancer (19%), bronchitis (18%), and pneumonia (16%) accounted for most causes of hemoptysis. In contrast to older studies, active tuberculosis was a rare finding (1.4%). Bronchiectasis and bleeding diathesis were major causes of moderate to severe hemoptysis while bronchitis and lung cancer were commonly associated with milder degrees of bleeding. CT scan was the most sensitive diagnostic test when employed alone, with a positive yield of 67%. However, it failed to locate at least three cases of lung cancer. When combining a CT study together with a bronchoscopy, the positive yield increased to 93%. The mortality rate for patients with mild to moderate hemoptysis was low (2.5% and 6%, respectively), while patients with massive hemoptysis had high mortality rates (38%). Patients with lung cancer or bleeding diathesis had higher mortality rates compared with the rest of the cohort. Conclusions Hemoptysis is a common symptom with a good prognosis in most cases. However, patients exhibiting massive bleeding or those with lung malignancy and patients with bleeding diathesis had a poorer prognosis. Patients older than 50 years with a positive smoking history need an extensive evaluation and follow-up to exclude lung carcinoma. The combined use of bronchoscopy and chest CT has the best yield in evaluating hemoptysis.

Heat stroke is a rare clinical phenomenon, characterized by systemic heat and cytokine-induced organ damage. Permanent neurological deficits rarely develop following heat strokes, and cerebellar dysfunction predominates among these rare cases. We report a case of severe heat stroke with recovery from severe multiorgan failure but with persistent neurological manifestations. These included a combination of resolving right-sided signs as well as persistent frontal subcortical dysfunction, with minimal, if any, cerebellar involvement. This combination and the absence of cerebellar involvement is an unusual neurological sequelae of heat stroke.

Category specific naming impairment was described mainly after cortical lesions. It is thought to result from a lesion in a specific network, reflecting the organization of our semantic knowledge. The deficit usually involves multiple semantic categories whose profile of naming deficit generally obeys the animate/inanimate dichotomy. Thalamic lesions cause general semantic naming deficit, and only rarely a category specific semantic deficit for very limited and highly specific categories. We performed a case-control study on a 56-year-old right-handed man who presented with language impairment following a left anterior thalamic infarction. His naming ability and semantic knowledge were evaluated in the visual, tactile and auditory modalities for stimuli from 11 different categories, and compared to that of five controls. In naming to visual stimuli the patient performed poorly (error rate>50%) in four categories: vegetables, toys, animals and body parts (average 70.31+/-15%). In each category there was a different dominating error type. He performed better in the other seven categories (tools, clothes, transportation, fruits, electric, furniture, kitchen utensils), averaging 14.28+/-9% errors. Further analysis revealed a dichotomy between naming in animate and inanimate categories in the visual and tactile modalities but not in response to auditory stimuli. Thus, a unique category specific profile of response and naming errors to visual and tactile, but not auditory stimuli was found after a left anterior thalamic infarction. This might reflect the role of the thalamus not only as a relay station but further as a central integrator of different stages of perceptual and semantic processing.

Controversy exists about whether depression following stroke represents a biologically mediated change or a psychological reaction to the deficits. We present a patient with an acute isolated personality change and depression following a lacunar infarct of the left internal capsule, who was unaware of his affective change. His anosognosia for the depression, which was abrupt and not accompanied by cognitive impairments, suggests that post stroke depression, at least in this case, is better explained by a biological than a psychological model.

Background Acute disseminated encephalomyelitis (ADEM) is a usually monophasic demyelinating disorder of the central nervous system. Recurrences pose a diagnostic challenge because they can be overlooked or suggest an alternative diagnosis. Objective To examine the frequency, nature, and outcome of recurrent ADEM. Design Review of the medical records of patients diagnosed in our institution as having ADEM between January 1, 1983, and May 31, 1998. Recurrences were defined as appearance of new symptoms and signs at least 1 month after the previous episode. Results Five (24%) of 21 patients with ADEM developed recurrent disease episodes. In all, diagnosis was confirmed by brain biopsy. One patient had 4 disease episodes, 2 had 3, and the other 2 each had 2. Recurrence appeared 1.5 to 32 months after initial presentation and involved the same brain territory in 6 of 9 recurrences in 3 of 5 patients. In 2 patients, recurrences included neuropsychiatric signs. A good response to corticosteroid therapy was observed in 10 of 13 of treated ADEM attacks: in 3 of the 4 treated initial events and in 7 of 9 recurrences. Conclusions Recurrent ADEM may be more prevalent than previously recognized. Patients who relapse tend to have more than 1 recurrence that usually involves, clinically and radiologically, a brain territory that was affected before and can simulate a space-occupying lesion that requires histologic diagnosis. Neuropsychiatric features may be the main presentation of a relapse. Since recurrent ADEM is a corticosteroid-responsive condition, awareness and early diagnosis are mandatory.

Coital headache or headache associated with sexual activity as classified by the International Classification of Diseases , revision 101 are characterized by abruptly appearing headaches occurring just before or at orgasm.2 It can be secondary to a structural lesion such as a vascular malformation, but is usually of a benign nature. The pathophysiology of benign coital headache is not clear, though an association with migrainous headache is noted.2,3⇓ Coital headache as a side effect of pharmacologic agents and chemical compounds has been reported with other medications such as birth control pills.3 Here we report a case of coital headache induced by amiodarone therapy. A 52-year-old, right-handed man presented to our …

We prospectively evaluated herpes zoster patients during the acute phase of the disease for central nervous system involvement. Of 24 patients with spinal zoster, 13 (54%) had spinal cord abnormality, which was asymptomatic in 12 of the 13. Age but not lack of acyclovir treatment was associated with such involvement. In all but 2, neurological involvement resolved within 6 months. Although the mechanism responsible for the neurological abnormalities is unknown, findings may support the hypothesis that zoster is associated with spread of viral infection into the spinal cord and therefore support the possibility that zoster is due to active viral replication in the ganglion.

In Stanley Kubrick's movie Dr Strangelove, the main character is described as "erratic" and displays a bizarre movement disorder. His right hand seems to be driven by a will of its own, at times clutching his own throat and at other times raising into a Nazi salute. Dr Strangelove must try to restrain this wayward limb with his left hand. Bizarre as this fictional character is, a similar movement disorder can occur in neurologic disease. The complex phenomenon associated with this disorder falls under the rubric of alien hand syndrome. This syndrome is characterized by a limb that seems to perform meaningful acts without being guided by the intention of the patient. Patients find themselves unable to stop the alien limb from reaching and grabbing objects, and they may be unable to release these grasped objects without using their other hand to pry open their fingers. These patients frequently express astonishment and frustration at the errant limb. They experience it as being controlled by an external agent and often refer to it in the third person. This article outlines the origins of the terminology used in describing this syndrome, early observations, and studies regarding its functional neuroanatomy.

Objective To investigate the incidence and characteristics of patients with structural central nervous system (CNS) lesions and cerebrospinal fluid oligoclonal IgG bands. Design A retrospective study. Method The medical records of patients with cerebrospinal fluid oligoclonal IgG bands were evaluated for the presence of structural CNS lesions, their location and cause, and for clinical characteristics. Setting Cerebrospinal fluid oligoclonal IgG bands were examined in the Neuroimmunology Laboratory, Hadassah University Hospital, Jerusalem, Israel. Patients Two hundred seventy of 570 patients with positive cerebrospinal fluid oligoclonal IgG bands were available for analysis. Twenty patients had structural CNS lesions. Results Twenty (7.5%) of the 270 patients had structural CNS lesions: 3 patients had spinal arteriovenous malformation; 5 patients had tumors; 9 patients had compressive cervical myelopathy. Traumatic leukomalacia, Arnold-Chiari malformation type 1, and CNS hemosiderosis were present in 1 patient each. In 2 patients (1 patient with recurrent meningioma and 1 patient with posttraumatic encephalomalacia) the presence of a structural CNS lesion was followed by the development of multiple sclerosis. In all 3 patients with spinal arteriovenous malformation, oligoclonal IgG identification prolonged the time to diagnosis and therapy, which varied from a few weeks to 3 years. Conclusions Structural CNS lesions, responsible for the neurological disorder, were present in 20 patients (7.5%) with cerebrospinal fluid oligoclonal IgG bands. The mechanism underlying oligoclonal IgG presence in spinal arteriovenous malformation and the coexistence of multiple sclerosis and structural CNS lesions is unknown, but may be related to recurrent tissue damage with repeated presentation of CNS antigens to the immune system.