Search

Objective: Catatonic disorder due to general medical condition must be excluded in psychiatric patients presenting with this movement disorder. This report emphasizes the association of catatonia with multiple sclerosis. Method: A patient with catatonia, psychotic depression, and the subsequent diagnosis of multiple sclerosis is described and the literature reviewed. Results: Mutism, immobility, cataplexy, waxy flexibility, and other aspects of catatonia occur in multiple sclerosis, usually as a consequence of a severe mood disorder and extensive cerebral demyelination. These symptoms may be the presenting manifestations of multiple sclerosis. Conclusions: A high index of suspicion for neurological disease is indicated in patients with new-onset catatonia. Neuroimaging and other studies may reveal underlying demyelination requiring specific therapy.

In the absence of depression, crying spells associated with neurological disease usually result from pseudobulbar palsy or, more rarely, from crying seizures. To our knowledge, there are no prior reports of crying spells heralding or signifying a transient ischaemic attack. We report on a patient with prominent cerebrovascular risk factors who had a transient episode of intractable crying and focal neurological findings. The patient was a 55 year old right handed man who presented with acute, uncontrolled crying spells followed by left sided paraesthesias. Around 6 00 am he awoke with a diffuse, pressure headache and suddenly started crying for no apparent reason. There was no accompanying feeling of sadness. This crying, which involved lacrimation and “sobbing,” abruptly ceased after 5 minutes. Within 30 minutes of his initial crying spell, his headache had resolved but he became aware of numbness over his left face and numbness and pain in his left neck and arm. The numbness was not progressive, and the patient did not complain of …

Laughter is a particularly human behavior. Neuropsychiatrists are faced with disorders of laughter, yet the nature of this behavior and its disturbances remains obscure. The authors report an unusual patient with involuntary and unremitting laughter for 20 years and review the literature. The nature of laughter suggests that it has a unique role in human communication, particularly in the social exploration of incongruous information. The disorders of laughter suggest a neuroanatomical circuitry that includes the anterior cingulate gyrus, caudal hypothalamus, temporal-amygdala structures, and a pontomedullary center. Treatment includes the use of antidepressant and antimanic agents for disorders of laughter.

SIR: Person misidentification syndromes usually involve the misperception of familiar persons or a loss of familiarity for normally perceived ones. We report a unique patient who developed the opposite, a sense of hyperfamiliarity for unfamiliar persons after a hemorrhage in the left anterior cingulate cortex (ACC).

Received April 8, 1997; revised July 8, 1997; accepted July 21, 1997. From the Department of Neurology and Psychiatry & Biobehavioral Sciences, University of California at Los Angeles; and the Neurobehavior Unit, West Los Angeles Veterans Affairs (VA) Medical Center. Address reprint requests to Dr. Mendez, Neurobehavior Unit (691/116AF), West Los Angeles VA Medical Center, 11301 Wilshire Blvd., Los Angeles, CA 90073. E-mail: mmendez@UCLA.edu. Copyright 1998 The Academy of Psychosomatic Medicine. The belief that epilepsy is linked with violence has existed since ancient times. This belief peaked in the 19th century when the criminologist Cesare Lombroso promoted the association of epilepsy with aggressive, sociopathic tendencies. Modern studies reject the notion that most persons with epilepsy are prone to violence; however, epidemiological studies reveal a greater occurrence of violence among those with seizures as compared with those without seizures. Most of this violence occurs during seizure-free periods and results from associated factors such as poor impulse control, cognitive impairments, or psychopathology. Although uncommon, violent acts may also result directly from seizures. Aggressive behaviors can occur as seizure prodrome, as ictal behaviors, as violent automatisms, or as part of the postictal confusional period. These violent acts are usually not well organized or specifically directed. Epileptic patients rarely have purposeful, organized violence as a direct manifestation of seizures or ictal discharges. I report the case of a rare patient with directed aggression as a postictal phenomenon and review the literature on epilepsy and violence. Case Report

Stereotypic movement disorders (SMD) such as head-banging, which are common among children with mental retardation or pervasive developmental disorders, may also occur in intellectually normal adults. We report a 27-year history of daily head-banging with self-injury in a 49-year-old man with normal cognition. The patient had no personal or family history of Tourette's syndrome, tic disorder, obsessive-compulsive disorder (OCD), or mental retardation. The frequency of his stereotypical head-banging increased with anxiety, loud noises with startle, and boredom. He reported a sense of pleasure from his head-banging, and the frequency of this behavior decreased when he was treated with the opioid antagonist naltrexone. Although not diagnostic, the self-stimulatory or pleasurable component of head-banging, body-rocking, thumb-sucking, and other SMD may help distinguish them from tics, Tourette's syndrome, OCD, and deliberate self-harming behavior. This report reviews the disorders associated with SMD and discusses the potential mechanisms for these behaviors. The treatment of SMD includes drugs that work through opioid, serotonergic, or dopaminergic systems.

This study investigated the cognitive estimations test (CET) in patients with frontotemporal dementia (FTD) and Alzheimer's disease (AD). The CET consists of questions which reflect judgment and reasoning. FTD patients, who have predominant frontotemporal atrophy, may give more extreme estimates on the CET compared to AD patients, who have greater temporoparietal pathology. The CET was administered to 31 FTD and 31 AD patients of comparable dementia severity plus 31 normal elderly controls. Both dementia groups gave significantly more extreme estimates on the CET than did the controls, and, contrary to expectations, AD patients gave more extreme estimates than did FTD patients. Extreme CET scores correlated with poor savings and calculations scores on neuropsychological tests. In conclusion, the CET may be particularly impaired in AD because it reflects impaired memory and numerical ability as well as disturbed judgment and reasoning. The CET may also be helpful in discriminating some patients in moderate stages of AD from those with FTD.

Frontotemporal dementia (FTD) is a common neurodegenerative dementia syndrome. Compulsive behaviors frequently occur in FTD and may be presenting symptoms of this disorder. This study evaluated compulsive behaviors as presenting symptoms in 29 patients with FTD compared to 48 patients with Alzheimer's disease (AD) enrolled in the UCLA Alzheimer's Disease Center. The FTD patients met the Lund and Manchester criteria for FTD and had predominant frontal hypoperfusion on single-photon emission computer tomography neuroimaging. The AD patients met National Institute of Neurological and Communicative Disorders-Alzheimer's Disease and Related Disorders criteria for clinically probable AD. Compulsive behaviors occurred in 11 FTD patients (38%) versus 5 AD patients (10%) (χ2 = 6.73, P < .01). This difference persisted after controlling for the younger age of the FTD group. There was a range of compulsive behaviors, with the most frequent being repetitive checking activities. Compulsive behaviors are common presenting symptoms among FTD patients and may result from an inability to inhibit urges to perform compulsive movements from damage to frontal-striatal circuits.

Disturbances in language and communication are prominent symptoms in dementia. Many dementing illnesses lead to aphasia, others primarily impair verbal fluency or result in motor speech disturbances such as dysarthria. This chapter discusses language and communication disturbances that occur in non-Alzheimer's dementias, including related abnormalities in cognition, aspects of neuropathology, and neuroimaging. The language deficits in vascular dementia (VaD), frontotemporal dementia (FTD) (e.g., Pick's disease), Huntington's disease (HD), progressive supranuclear palsy (PSP), Creutzfeldt-Jakob disease (CJD), and primary progessive aphasia (PPA) are included. Implications for future research are also noted.

Fabry's disease, a rare X-linked disorder of glycosphingolipid metabolism, can present as an insidious dementia in middle or later life. This genetic disorder produces a deficiency of alpha-galactosidase A which results in the deposition of glycosphingolipids in blood vessel walls in the brain as well as in the kidney, heart, peripheral nerves, and other organs. Among the cerebrovascular manifestations of this disorder is a vascular dementia from involvement of multiple small penetrating blood vessels. Fabry's disease is a consideration in the workup of an otherwise unexplained vascular dementia, particularly in males less than 65 years of age.

Frontotemporal dementia (FTD) is a common neurodegenerative dementia that can be difficult to distinguish clinically from Alzheimer's disease (AD).The differential distribution of pathology in FTD and AD predicts the presence of differential cognitive features on mental status examination. We compared 39 FTD patients with 101 AD patients on the Consortium to Establish a Registry in AD examination supplemented by cognitive areas from the Neurobehavioral Cognitive Status Examination. The FTD patients were diagnosed using noncognitive clinical and neuro-imaging criteria and were comparable to the AD patients in terms of gender, educational level, and dementia severity ratings. The FTD patients performed significantly better than the AD patients on constructions and calculations. These findings were at the lower limits of normal for older normal controls and persisted after covarying for younger age and higher Mini-Mental Status Examination scores in the FTD group. In addition to personality and neuroimaging features, relatively preserved performance of elementary drawings and calculations in FTD suggests additional features for distinguishing FTD patients from comparably demented AD patients.NEUROLOGY 1996;47: 1189-1194

Forced thinking is an incompletely understood and rarely described epileptic aura.We studied three patients with forced thinking from left frontal lesions, two neoplastic and one vascular. All three experienced repetitive, intrusive thoughts at the onset of seizures. Their forced thinking was associated with the desire to vocalize, orobuccal movements, and speech arrest. The episodes occurred with other ictal manifestations and responded to antiseizure therapy. These patients suggest that epileptic forced thinking is a heterogeneous phenomenon; forced thinking from left frontal lesions is a manifestation of expressive language and is distinct from experiential thoughts arising from temporal limbic foci.NEUROLOGY 1996;47: 79-83

Objective: There are few direct comparisons between early-onset Alzheimer9s disease (EAD) and late-onset Alzheimer9s disease (LAD) patients. This study aims to compare patients with EAD vs. LAD on neuropsychological and neuroimaging measures and identify distinguishing features between the two. Background EAD, with an onset before age 65, has been overshadowed by the more common LAD. Yet, the literature suggest that there are clinical differences between these two forms of the disease. Patients with EAD patients may have more focal neurocognitive deficits and a more aggressive and symmetrical progression than patients with LAD. Design/Methods: Participants included 21 EAD and 24 LAD patients who met criteria for clinically probable AD and had comparable levels of disease progression and severity. The patients underwent neurological examinations, neuropsychological testing, and resting state FDG-PET of the brain. Demographic neuropsychological, neuropsychiatric, and functional variables were compared using Chi Square analyses, independent samples t-tests, and ANCOVA. FDG-PET data was compared using voxel-based analysis with statistical parametric mapping (SPM). Results: There were significant neuropsychological differences between the two groups: the EAD patients were more impaired in response-inhibition and auditory working memory (measures of executive functions), whereas the LAD patients were more impaired on confrontation naming. Caregivers reported greater prevalence of irritability and anxiety in EAD vs. LAD, and EAD patients needed more assistance with initiating grooming, meal preparation, writing, and dressing tasks compared to LAD patients. On SPM PET analyses, the EAD group had greater focal hypometabolism in both frontal and parietal regions compared to LAD. Conclusions: This study shows clear differences between EAD and LAD. Patients with EAD have more focal and frontal dysfunction when compared to LAD patients, and this is reflected in executive function disturbances. Although these findings are partially at variance with prior research, they confirm the presence of clinical differences between these two forms of AD. Supported by: Merit Review, Veterans Affairs. Disclosure: Dr. Kaiser has nothing to disclose. Dr. Liu has nothing to disclose. Dr. Melrose has nothing to disclose. Dr. Jimenez has nothing to disclose. Dr. Monserratt has nothing to disclose. Dr. Sultzer has received personal compensation for activities with Bristol-Myers Squibb Company, Forest Laboratories, Inc., Pfizer Inc, Janssen Pharmaceutica, Abbott Laboratories, Inc., and AstraZeneca. Dr. Sultzer has received research support from Pfizer Inc, and Forest Laboratories. Dr. Mendez has nothing to disclose.

Objective: To investigate neurological differences between Non-familial EAD and PSEN1, the most common FAD, as markers for FAD. Background Although familial Alzheimer9s disease (FAD) is an Early onset AD (EAD), most patients with EAD do not have a familial disorder. Recent guidelines recommend obtaining genetic testing only in those EAD patients with 2 first-degree relatives. However, some patients with FAD may lack a known family history or other indications for suspecting FAD but might nonetheless be FAD mutation carriers and the results of such testing helpful to clinicians and families. Design/Methods: Retrospective chart review of a large University-based cohort of patients with non-familial early-onset AD (n=75) and with PSEN1-related AD (n=31). Results: There were group differences in age of onset, with PSEN1 mutation carriers being significantly younger, and in disease severity/duration (p Conclusions: In addition to a much younger age of onset, clinicians may be able to recognize FAD patients with PSEN1 mutations by specific abnormalities on the neurological history and examination. In some contexts, these findings should lead to genetic counseling and appropriate recommendations for obtaining PSEN1 testing or other genetic tests for FAD. Supported by: PHS K08 AG-22228, California DHS #04-35522, Alzheimer9s Disease Research Center Grant P50 AG-16570 from the National Institute on Aging, the Easton Consortium for Alzheimer9s Disease Drug Discovery and Biomarkers, the Sidell Kagan Foundation, the Shirley and Jack Goldberg Trust and Veteran9s Affairs Merit award. Disclosure: Dr. Joshi has nothing to disclose. Dr. Lee has nothing to disclose. Dr. Ringman has received personal compensation for activities with Takeda Pharmaceuticals as a scientific advisory board member.Dr. Ringman has received research support from Wyeth, Elan, Janssen, Bristol-Myers Squibb, Medivation, and Pfizer Pharmaceuticals. Dr. Juarez has nothing to disclose. Dr. Mendez has nothing to disclose.

Objective: This study investigated changes in functional health and well-being, cognition, and functional neuroimaging among Iraq/Afghanistan veterans who sustained pure blast-force mild traumatic brain injury (mTBI), as compared to those who sustained pure blunt-force mTBI. The aim was to document post-concussion consequences and associated changes in cerebral metabolism from mTBI due to blast forces. Background Injuries from blast forces are a hallmark of the conflicts in Iraq and Afghanistan and may cause undetected neurological dysfunction from mTBI. The effects of primary blast overpressure on the brain have been difficult to clarify because of complications, such as secondary and tertiary blast effects and comorbid post-traumatic stress disorder (PTSD). Design/Methods: Inclusion criteria included presence of pure blast or blunt injury, absence of PTSD, and between 6-36 months post-trauma. The Blast Group (n=12) and the Blunt Group (n=12) underwent assessments of well-being, cognition, and resting fluorodeoxyglucose positron emission tomography (PET). Results: There were no group differences on mTBI-related variables, the Rivermead Post-Concussion Questionnaire (RPQ), or the SF36-V Health Survey. Both groups, however, did worse than published norms on the RPQ and SF36-V, and the Blast Group had additional worse SF36-V subscale scores on Bodily Pain and Social Functioning. Compared to the Blunt Group, the Blast Group had poorer scores on the Paced Auditory Serial Addition Test (PASAT) and greater PET hypometabolism in the right superior parietal region. Only the Blast Group had significant correlations of their RPQ, SF36-V Mental Composite Score, and PASAT scores with specific regional metabolic changes. Conclusions: Together these results suggest that pure blast force mTBI has post-concussion consequences. Blast mTBI may cause deficits in well-being, possibly from an altered bodily self-image, and in attentional control. These changes are associated with regional changes in cerebral metabolism, particularly hypometabolism in the right hemisphere. Supported by: Department of Veteran9s Affairs Grant. Disclosure: Dr. Mendez has nothing to disclose. Dr. Licht has nothing to disclose. Dr. Berenji has nothing to disclose. Dr. Peppers has nothing to disclose.

Patients with epilepsy often have depressive disorders. This association may be particularly prominent in secondary epilepsy from a left hemisphere lesion. Among 1611 outpatients with epilepsy 272 patients were identified whose seizures originated from a structural brain lesion other than mesial temporal sclerosis. Sustained depressive disorders had occurred in 25 (9%) of these patients with secondary epilepsy. The depressed patients were compared with the remaining patients without depression with regard to location of lesion laterality and seizure variables. The only group difference was unilateral left hemisphere lesions in 58% of the patients with depression compared with only 21% of the non-depressed patients (chi 2 = 10.4, p = 0.006). This finding supports the idea of a relation of depression with epileptogenic lesions in the left hemisphere.

Interictal violence among epileptic patients could result from factors other than epileptiform activity. We characterized 44 patients who presented for psychiatric evaluation because of violent behavior. Most violent acts consisted of verbal or minor physical aggression. Twenty (45%) of these patients met criteria for a schizophrenic disorder, and one committed murder during a paranoid schizophrenic relapse. In addition to schizophrenia, the violence patients had significantly more mental retardation when compared with 88 age- and sex-matched epileptic patients without prior violent behavior. However, violent and nonviolent patients did not differ on seizure variables such as type and frequency of seizures, auras, electroencephalographic changes, epilepsy age of onset, or anticonvulsant therapy. These findings suggest that interictal violence is associated more with psychopathology and mental retardation than with epileptiform activity or other seizure variables.

Among epileptic patients, personality disorders may be associated with specific seizure manifestations. In an epilepsy clinic, we identified 42 idiopathic epilepsy patients diagnosed with various DSM-III-R personality disorders, including borderline, atypical or mixed, explosive, and dependent. When compared with 42 age- and sex-matched epileptic control subjects on six seizure variables, the personality disorder group had more patients with epileptic auras (P = 0.001), particularly "cephalic" auras, and fewer with secondarily generalized tonic-clonic seizures (P < 0.05). These findings suggest that the experience of epileptic auras contributes to the development of personality disorders, especially when auras are not masked by secondarily generalized seizures.

The misidentification of a familiar person or of oneself may occur as a complication of dementia. Seven patients experienced alterations of the sense of familiarity for a familiar object or place, for the misidentified person in a novel role, for personal characteristics, or for unfamiliar events as familiar. Five of these had persecutory delusions. These cases suggest that person misidentification in dementia begins with an altered sense of familiarity for a familiar person from a mismatch of new perceptions with past memories. They are sustained by paranoid elaboration or confabulatory rationalisation of a double.

Objective: To evaluate the ictal and psychiatric aspects underlying the high risk of suicide among epileptic patients. Method: We surveyed the records of 1611 epileptic patients seen in a neurology clinic during an eight-year period, found four who died by suicide, and characterized their ictal and psychiatric features. Results: All four epileptic patients had partial complex seizures and temporal lobe foci. Two had an associated paranoid schizophrenia with suicidal ideation, and good or improving seizure control at the tine of their deaths. Another patient killed himself during a brief psychotic episode associated with increasing epileptiform discharges. The fourth patient had ictal depression and committed suicide during a flurry of partial complex seizures. Conclusions: These findings suggest that the high suicide rate among epileptic patients has a greater association with psychotic behaviors and psychic auras than with major depression or the psychosocial burden of being epileptic. We further review other reported risk factors for suicide in epilepsy.

In this issue of Neurology , Kertesz et al. integrate neurobehavioral, genetic, and pathologic data to analyze a dominantly inherited disease phenotype that the authors refer to as Pick complex or frontotemporal dementia (FTD).1 This study was promoted by the discovery that mutations in the tau gene cause FTD and parkinsonism linked to chromosome 17 (FTDP-17).2-7 The FTDP-17 phenotype encompasses seemingly heterogeneous, inherited clinicopathologic entities such as pallido-ponto-nigral degeneration, familial multiple system tauopathy, hereditary-dysphasic-disinhibition-dementia, familial progressive subcortical gliosis, and the disinhibition-dementia-parkinsonism-amyotrophy complex.8 These phenotypes are all genetically linked to the tau locus. Not surprisingly, variations in the pathology of FTDP-17 have been detected by ultrastructural and immunocytochemical analysis of aberrant neuronal and glial proteins (e.g., tau, α-synuclein, neurofilament, beta-amyloid, human glial fibrillary acid protein, the prion protein, vimentin, and ubiquitin). Some, but not all, of these variations are due to the location of tau gene mutations and their effect on …

Although patients with multi-infarct dementia (MID) may have greater spontaneity than those with dementia of the Alzheimer's type (DAT), conventional neuropsychological tests often fail to distinguish between these two dementias. We studied 18 patients with MID and compared them to 18 comparably demented patients with DAT and 18 normal elderly controls on conventional structured tests and two unstructured tasks, a 3-minute verbal description of the Cookie Theft Picture and the Lezak Tinker Toy Test. On the structured tests, the only significant difference between the two dementia groups was worse performance by the DAT patients on several memory measures. On the unstructured tasks, the MID group had significantly fewer words/minute and constructional assemblages. These results suggest that unstructured tasks help distinguish patients with MID from those with DAT, and that MID patients have decreased spontaneous behavior and initiation, possibly reflecting frontal-subcortical pathology and compromised executive abilities.

In the absence of pathognomonic clinical features, the clinical diagnosis of Alzheimer's disease (AD) remains one of exclusion of other dementias. We investigated the clinical diagnoses among 394 neuropathologically confirmed AD cases in a dementia brain bank. Most patients were correctly diagnosed as AD (348 or 88%). Among the misdiagnosed patients, AD was mistaken for a primary depressive disorder in 14, multi-infarct dementia in 13, Parkinson's disease in nine, and alcoholic dementia in four. The number of misdiagnosed AD patients did not differ between physician specialties but was greater among AD patients with agitation, depression, paranoia, or delusions. This retrospective study suggests that the diagnostic sensitivity for AD is high among a cross-section of practicing physicians and that an important factor in mistaking AD for another illness is unfamiliarity with the potential psychiatric symptoms of AD. ( J Geriatr Psychiatry Neurol 1991;4:26-29).

On the surface, reviewing a dictionary seems like a relatively straightforward task. The book is an extensive compilation of terms used in psychology and psychiatry. The dictionary provides a comprehensive listing of the English–Spanish translation equivalents that can be useful to psychologists and psychiatrists working in increasingly bilingual environments. This book could facilitate professional communications across the English–Spanish divide.

A retrospective chart review was undertaken to assess the prevalence of specifically defined psychiatric symptoms in 217 outpatients with clinically probable Alzheimer's disease. A weak but significant correlation was found between Alzheimer's disease and suspiciousness and paranoia (reported by 35.5% of patients), delusions (30%), and formed visual hallucinations (18.4%). Mild depressive symptoms, such as sad affect, hopelessness, and helplessness were reported by 40.6% of patients, anxiety and fearfulness by 30.9%, and aggressive acts by 24.9%. Psychotropic medications used to manage these symptoms included antipsychotics, in 11.5% of patients, anxiolytics, in 9.2%, and antidepressants, in 7.4%.

In Reply. —Leviton and colleagues question our choice of a control group. 1 As noted by Kelsey et al, 2 recognized authorities in the area of comparative studies, "Choice of the most appropriate control group is one of the most difficult and controversial aspects of study design... [and] in many situations no single control group is obviously best. In such situations it is helpful if... more than one case-control study of an area of interest is undertaken by different investigators using different types of control groups...." 2 The approach suggested by Leviton et al is one way of investigating the problem, but not the only way as they aver. If controls are selected on the basis of epilepsy as Leviton et al suggest, there is no way to determine whether psychopathologic factors differ in epileptic and nonepileptic suicide attempters, nor can the effect of chronic psychosocial stressors related to having

The clinical recognition of Pick's disease depends on its differentiation from Alzheimer's disease (AD). To identify distinguishing clinical features, we reviewed the clinical records of 21 patients with pathologically confirmed Pick's disease and matched them by sex, age of onset, and duration of dementia with 42 patients having pathologically confirmed AD. In the absence of temporal or frontal lobar atrophy on CTs, all the Pick patients and none of the AD patients had three of five clinical features: presenile onset (before age 65), an initial personality change, hyperorality, disinhibition, and roaming behavior. In addition, the Pick patients had a tendency toward reiterative and other speech disturbances. These findings suggest that Pick patients are potentially distinguishable from AD patients on the basis of clinical manifestations.

We investigated potential risk factors for Alzheimer's disease (AD) in a clinicopathologic study of 407 patients with definite AD, 100 non-Alzheimer dementia patients, and 50 normal subjects. The AD patients had more first-degree relatives with dementia than the non-AD dementia group (odds ratio of 1.85, 95% confidence interval of 1.07-3.20) or the normal elderly (odds ratio of 3.60, 95% confidence interval of 1.50-8.64) but did not have significantly more head injuries, medical and psychiatric illnesses, or relatives with Down's syndrome. The AD patients with a family history of dementia had their dementia at a later age than those without an affected relative. These findings indicate a familial risk for AD that is greater than for other dementing illnesses and has age-related penetrance. This study does not support other putative risk factors for AD such as head trauma and familial Down's syndrome.