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PubMed1988

Neurologic fragments. I. Clinical observations in demented patients.

Some 28 clinical observations of behavior, mainly in demented patients, have been singled out for brief description and comment. Most are familiar, and the aim of the presentation is to identify and codify them, complementing formal psychological tests. Several have been given names--eg, insistence, balking, dread of being alone, the obligatory alibi, lability of temper, amphigory, reaching out, etc. Others draw attention to and characterize fragments of behavior--eg, ambient echolalia, periodic repetition of questions and comments, failure to recognize the spouse, turning the back, etc. Finally, a few clinical phenomena are commented upon.

Alzheimer Disease↗
PubMed1994

Familial progressive subcortical gliosis.

We report clinical and pathologic findings from two kindreds afflicted with a familial form of progressive subcortical gliosis. The disorder segregated as an autosomal dominant trait. Onset was in the presenium and the course was slowly progressive. Affected individuals initially manifested personality change, degeneration of social ability, disinhibition, psychotic symptoms, memory impairment, or depression. Later, all developed progressive dementia, frequently associated with verbal stereotypy, decreased speech output, echolalia, or manifestations of the human Klüver-Bucy syndrome. Terminal clinical manifestations included profound dementia, frequently with mutism, dysphagia, and extrapyramidal signs. Autopsy of seven end-stage patients revealed generalized cerebral atrophy, predominantly involving the white matter of the frontal and temporal lobes. Microscopically, prominent fibrillary astrocytosis was present in the subcortical white matter and in the subpial and deep layers of the overlying cerebral cortex. These changes were most pronounced in the frontal and temporal lobes, especially in the cingulate gyri and insulae. Mild cortical neuronal loss accompanied the gliosis, but no myelin loss was evident. The claustra and substantia nigra also showed severe astrocytosis and degenerative changes. Amyloid deposits and neuronal cytoskeletal inclusions were absent.

Aged↗
PubMed2004

Intentional topiramate ingestion in an adolescent female.

OBJECTIVE: To describe an intentional topiramate ingestion by an adolescent and warn of the potential for topiramate abuse. CASE SUMMARY: A 17-year-old female intentionally ingested approximately eight 100-mg topiramate tablets for the purpose of "getting high." Soon after ingestion, she was found at school obtunded and nonresponsive. Upon transfer to the emergency department, she became combative and aggressive with evolving neurologic abnormalities including incoherence, confusion, disorientation, and significant speech impairments including echolalia. Approximately 24 hours after ingestion, the patient had completely recovered without requiring specific treatment or experiencing sequelae. DISCUSSION: The clinical effects following acute topiramate intoxication appear consistent with the drug's known pharmacologic properties. There are few other reports of topiramate ingestions and most cases have had mild outcomes. CONCLUSIONS: Due to the multifactorial effects topiramate may have upon the central nervous system and its anorectic effect, abuse of this drug by adolescents should be considered upon presentation of an adolescent with mental status changes.

Adolescent↗
PubMed2000

Counseling parents regarding prognosis in autistic spectrum disorder.

A triaxial model for autistic spectrum disorder (ASD) is presented, incorporating age, degree of intelligence, and severity of autistic features. As the name implies, ASD can vary in degree of expression from minimal to profound. Furthermore, the symptoms of ASD change in predictable ways with the passage of time. For example, echolalia during early childhood may be replaced by verbal literalism and difficulty with verbal humor during later childhood or adolescence. The prognosis for children with ASD is governed by the joint impact of the degree of expression of ASD and the degree of developmental delay, if any. All combinations of ASD and intellect are possible (ie, severe ASD plus severe mental retardation, severe ASD plus normal general intelligence, and so forth). The relationship among these 3 parameters-severity of ASD, level of general intelligence, and change in symptom expression over time, is represented schematically as a 3-dimensional graph. The utility of this graph as a counseling tool, and as the basis for future research on the prognosis of ASD are discussed.

Age Factors↗
PubMed1973

Some generalization and follow-up measures on autistic children in behavior therapy.

We have treated 20 autistic children with behavior therapy. At intake, most of the children were severely disturbed, having symptoms indicating an extremely poor prognosis. The children were treated in separate groups, and some were treated more than once, allowing for within- and between-subject replications of treatment effects. We have employed reliable measures of generalization across situations and behaviors as well as across time (follow-up). The findings can be summarized as follows: (1) Inappropriate behaviors (self-stimulation and echolalia) decreased during treatment, and appropriate behaviors (appropriate speech, appropriate play, and social non-verbal behaviors) increased. (2) Spontaneous social interactions and the spontaneous use of language occurred about eight months into treatment for some of the children. (3) IQs and social quotients reflected improvement during treatment. (4) There were no exceptions to the improvement, however, some of the children improved more than others. (5) Follow-up measures recorded 1 to 4 yr after treatment showed that large differences between groups of children depended upon the post-treatment environment (those groups whose parents were trained to carry out behavior therapy continued to improve, while children who were institutionalized regressed). (6) A brief reinstatement of behavior therapy could temporarily re-establish some of the original therapeutic gains made by the children who were subsequently institutionalized.

Journal Article↗
PubMed1996

Effects of left basal ganglia lesions on language production.

Three patients with lesions mainly confined to the left basal ganglia were studied with a series of neuropsychological and neurolinguistic tests. Two patients were nonfluent, whereas one presented with fluent spontaneous speech. All of them produced agrammatic sentences and lexical and semantic mistakes. Perserverations and echolalias were two further characteristic disorders of their speech production. The linguistic symptoms observed in these three patients suggest that the left basal ganglia play an important role (a) in regulating arousal and speech initiation, (b) in monitoring the semantic and lexical aspects of language, and (c) in switching from one linguistic element to the following during language production.

Aged↗
PubMed1999

[Tics disease (Gilles de la Tourette syndrome): clinical characteristics of 70 patients].

BACKGROUND: Tourette's syndrome is a childhood-onset hereditary neurobehavioural disorder believed to occur without geographical restrictions. Although there have been reports of this disorder worldwide just a few are from Latin America. AIM: To report a preliminary experience with a series of 70 patients and to review recent advances in this disorder. PATIENTS AND METHOD: We reviewed patients seen in pediatric and adult neurological clinics in Santiago, Chile, all of whom fulfilled clinical diagnostic criteria for Tourette Syndrome. RESULTS: Seventy patients were studied, 54 males (77.1%) and 16 females (22.8%), their mean age at first evaluation was 13.6 years (range 2-46). The mean age of onset of symptoms was 6.4 (range 2-20), the mean time of follow-up was 3 years. Fifty-eight patients showed simple motor tics (blinking, facial grimacing, shoulder shrugging), whereas dystonic tics like head jerking were seen in 38 patients, torticollis in 6 and oculogyric movements in 2. Complex motor tics like jumping, antics, trunk bending and head shaking were present in 16 subjects. Vocal tics were predominantly of the simple type: sniffing, throat clearing, blowing, and whistling. Complex vocal tics were seen in 12 patients, five cases showed palilalia, 3 echolalia and only six displayed coprolalia (8.5%). Tics were of mild to moderate severity in most patients. Obsessive-compulsive disorder was observed in 22.8% and attention deficit and hyperactivity disorder were present in 35.7%. Forty-five patients (64.2%) had a first degree relative with tics, nine patients (12.8%) had a family history of obsessive-compulsive disorder. The current evidence involving desinhibition of cortico-striatum-thalamic-cortical neuronal circuits in the pathogenesis of this disorder is analyzed. CONCLUSION: Our report supports the recognized clinical homogeneity and genetical basis of Tourette's syndrome regardless of geographical region and ethnic origin.

Adult↗
PubMed2000

[A case of multiple sclerosis with hypothalamic amenorrhea].

We present a 31-year-old woman of multiple sclerosis. At age 28, she was admitted with complaints of echolalia and a gradual onset of weakness affecting the right upper and bilateral lower limbs. Brain MRI showed high intensity areas in the bilateral frontal gyri, lobuli paracentralis, and left anterior thalamus. Although she had been in remission for 3 years, she developed dysesthesia of left upper and lower limbs. Cervical T2 weighted MRI showed a new high signal intensity lesion in the spinal cord from the C2 to C3 level. The combination of the cerebral, thalamic and spinal cord lesions with remission and excerbations allowed the diagnosis of clinically MS to be made. She suffered amenorrhea from the onset of her illness. Serum prolactin was within the normal range. The LH and FSH basal secretions were decreased and there were low delayed secretions of LH and FSH after intravenous injection of 100 micrograms LHRH. We consider that her amenorrhea was caused by the hypothalamic lesion, supported by MR findings of dilatation of the third ventricle.

Adult↗
PubMed2000

[A 57-year-old woman with progressive disturbance of gait and mental deterioration].

We report a 57-year-old woman with progressive gait disturbance and mental deterioration. She was well until March 1995, when she was 54 years of the age. At that time she noted a gradual onset of tremor and difficulty using her hand. Similar symptoms appeared in her right hands, and she visited another hospital, where 300 mg of levodopa and 7.5 mg of bromocriptine were prescribed. These medication did not help her symptoms. In the summer of 1996, she became to fall down easily. In September of the same year, she started to repeat the same words many times. She was unable to stop it. She was hospitalized to our service on January 25, 1997. On admission, she was alert but demented moderately; her Hasegawa dementia scale was 15/30. She showed palilallia, logoclonia, and echolalia. She showed constructional apraxia and questionable left-right disorientation. She had marked vertical gaze palsy with preserved oculocephalic response. She had masked face and small voice. Her gait was wide based with small steps. No muscle atrophy or weakness was noted. She showed only mild rigidity in the neck, but no rigidity was noted in the limb. No tremor was noted. She was bradykinetic. Deep tendon reflexes were symmetric and within normal limits. Laboratory findings on admission was unremarkable. MRI showed atrophy of the brain stem as well as cerebral cortical areas, particularly in the fronto-temporal region. Her hospital course was complicated with paralytic ileus and septicemia. She developed hypotension and pronounced dead on July 28, 1998. She was discussed in the neurological CPC. The chief discussant arrived at a conclusion that the patient had progressive supranuclear palsy and died of septic shock. All the participants wondered between PSP and CBD, but majority agreed with this diagnosis of the chief discussant. Only one thought that she might have had corticobasal degeneration rather than PSP, because of dementia, cortical atrophy in MRI, and lack of limb rigidity. Postmortem examination revealed cortical and brain stem atrophy. In the premotor cortex, marked astrocytosis and ballooned neurons were seen. Furthermore, astrocytic plaques were seen; this is considered to be pathognomonic for CBD. The substantia nigra showed marked neuronal loss and gliosis, but no neurofibrillary tangles or Lewy bodies were seen. Gliosis was also seen in the globus pallidus and in the medial thalamus. The pathologic diagnosis was corticobasal degeneration. This patient was very interesting case, in that the clinical manifestations appeared to be consistent with PSP, yet pathologic diagnosis was CBD. Lack of limb rigidity may be atypical for advanced PSP. In addition, palilalia appears to be more associated with CBD.

Brain↗
PubMedDate not supplied

[Formal thought disorders: French translation of the Thought, Language and Communication assessment scale].

This article proposes a french translation of Andreasen's Thought, Language and Communication (TLC) scale (Andreasen, 1979). This scale is widely used in current literature and remains a reference due to the fact that it has made it possible to establish a consensus with regard to formal thought disorders and has contributed to the operationalisation of the concept of dissociation. This scale consists of 18 items. Each item is clearly defined through the use of clinical examples, rated from 0 to 4 as a function of the intensity of the disorder (absent, slight, medium, severe, extreme). The interview conditions are also stated: free interview of minimum 10 minutes followed by a more structured interview. Some items of the TLC are taken directly from the SANS and SAPS. Their translation has been taken over from french translations already validated by Lecrubier and Boyer (1987). The others were translated within the department and have been verified by a native English speaker. The entirely of the translation has been verified by Andreasen. The metrological qualities of this french translation have been studied in a population of 107 schizophrenic patients who fulfilled all the DSM IV criteria: 73 males and 34 females, mean age 33.4 9 years, in or outpatients, all under neuroleptic treatment and all evaluated by an experienced clinician. Thirty one patients have been filmed to assess the interjudge reliability. The results indicate a high level of interjudge consistency (interclass correlation coefficient 0.96). The global score was 17 9.4. In the factorial analysis before rotation we observe a main factor that makes it possible to calculate a global score. The results of factor analysis of the TLC variables after rotation yield five factors that have an eigen value greater than 1. These five factors explain 66% of the variance. All items have a weight greater than 0.45. The first factor includes Poverty of content speech, Tangentiality, Derailment, Incoherence, Illogical thinking, Loss of goal and Perseveration. It reflects thinking disorganisation. The second factor includes Pressure of speech, Circumstantiality, Self reference and Poverty of speech (negative weight). This factor reflects verbal production. The third factor is composed of Clanging, Neologisms, Word approximation and Echolalia. This factor reflects verbal structure. The fourth factor is only composed of Stilted speech and the fifth one composed of Distractible speech and Blocking. These data have been compared to those reported in the literature: Andreasen in 1979 (113 patients: 32 suffering from manic disorder, 36 from depressive disorder and 45 schizophrenic disorder) and in 1986 (194 subjects: 94 controls, 25 suffering from manic disorder, 25 schizoaffective disorder and 50 schizophrenic patients), Harvey in 1992 (115 schizophrenic patients) and Peralta in 1992 (142 schizophrenic patients). Response levels for each item of the TLC french translation were very close to those found in the english versions. Differences in scores can be explained by clinical differences between populations studied. Factorial analyses also correspond well to such versions. In particular, after rotation, the three factorial subscores found representative of disorganisation, verbal production and verbal structure respectively are closed to those proposed by the english versions. In conclusion, the translation of Andreasen's Thought, Language and Communication (TLC) scale (Andreasen 1979) that we propose here therefore appears to exhibit metrological qualities sufficiently close to those reported in literature to permit its generalised use in France.

Adult↗
PubMedDate not supplied

[Current theories on the etiology and treatment of Gilles de la Tourette's disease].

Gilles de la Tourette syndrome is a condition marked by: (1) onset usually in childhood and adolescence, i.e. between 2 and 15 years of life; (2) violent facial tics and echolalia; (3) increased excitability and apathy; (4) progressive increase in symptoms intensity; (5) chronic course. This syndrome is threefold more frequent in men than in women. None hypothesis concerning its etiopathogenesis (genetic, organic, organic-functional, psychomotor, and mixed) does explain its origin. Many cases respond with some degree of relief to neuroleptics, carbamazepine, clonidine, and glucocorticosteroids. Neurosurgery and psychotherapy are also of value. Haloperidol is commonly considered the most effective in this syndrome.

Adolescent↗
PubMed2003

[Non convulsive status epilepticus: an heterogeneous disease with a difficult diagnosis. Report of 2 cases with unusual presentation].

Non convulsive status epilepticus is a heterogeneous condition consisting of very different electroclinical syndromes. It is difficult to make the diagnosis and identify common factors among patients. We report two cases with an unusual presentation. A 31 years old woman having discoid lupus presented with a prolonged exogenous psychosis that lasted two and half months, associated to echolalia. After the episode the patient remained with a severe frontal syndrome that could be the consequence of a non convulsive status epilepticus. A 60 years old woman with an epilepsy diagnosed at the age of 30, presented with recurrent episodes of aphasia. During one of these crises, the electroencephalogram showed continuous epileptiform activity.

Adult↗
PubMedDate not supplied

[Gilles de la Tourette's syndrome].

Gilles de la Tourette's syndrome (GTS) is characterized with motor and vocal tics, initiating before 21 years of age, lasting for over a year and are associated with diverse behavioral disorders. The study analyzes features of 12 our GTS patients with mean age at the onset of the disease of 12.0 years, while the exact diagnosis was established only after 9.5 years (2-33 years). In 11 out of 12 patients the disease started with motor or vocal tics, while in completely developed clinical picture of GTS the permanently present tics were associated with coprolalia (6 patients), echolalia, copropraxia (in 2 patients, respectively), attention deficits (9 patients), obsessive-compulsive disorders (8 patients) etv. The role of neuroleptic therapy was discussed according to our experience and other reported studies. In our study, haloperidol had good therapeutical effect in 64% of the treated patients.

Adolescent↗
PubMedDate not supplied

Progressive supranuclear palsy with widespread cerebral lesions.

A 51-year-old woman with no history of any familial neurological diseases initially presented with numbness in her extremities, slowing of movements, comprehension deficit, memory disturbance, dyscalculia, muscle rigidity, hyperreflexia, Parkinsonian gait, increasing disorientation, left-right disturbance, finger agnosia, alexia, acalculia, apraxia, aspontaneity, euphoria, gait disturbance, aphasia, echolalia, and in the terminal stage, mutism, contracture of lower extremities and cachexia. She died of bronchopneumonia at the age of 55. The brain showed widespread cerebral lesions, consisting of nerve cell loss and neurofibrillary tangles in the frontal, parietal and occipital cortex, demyelination and gliosis in the frontal, parietal and occipital subcortical white matter in addition to the typical pathological findings of progressive supranuclear palsy (PSP): severe neuronal loss with gliosis and neurofibrillary tangles (NFTs) in the subthalamic nucleus, globus pallidus and substantia nigra. In conclusion, we present a case of PSP with unusual clinical features (extrapyramidal signs, frontal and parietal lobe syndromes without ophthalmoplegia) and neuropathologically widespread cerebral lesions in addition to the typical pathological findings of PSP. The differential diagnosis of PSP and Alzheimer's disease and other degenerative disorders is discussed.

Alzheimer Disease↗
PubMed1991

Autistic children: diagnosis and clinical features.

Autism is one of the behaviorally defined developmental disorders of brain function. It has a variety of genetic and nongenetic etiologies, with etiology being unknown in the majority of children. Boys are more frequently affected than girls. Manifest in the preschool years, autism always affects sociability, communication, and the child's repertoire of activities and interests. Autism encompasses children with a broad range of severities and a variety of other signs of brain dysfunction. These include motor signs, notably stereotypies; abnormal responses to a variety of sensory stimuli; and disorders of affect and attention. A significant proportion of autistic children experience epileptic seizures and have abnormal EEGs. Neuroimaging, preferably magnetic resonance imaging, discloses abnormalities of brain development in a minority of autistic persons. The level of intelligence may range from profound mental deficiency to giftedness. The pattern of cognitive skills is likely to be uneven, typically with better nonverbal than verbal skills. In the preschool years, all autistic children have a developmental language disorder. Verbal expression may range from total lack of language to verbosity with echolalia; comprehension and language use are invariably impaired. While there is no specific pharmacologic agent to mitigate the fundamental disorder, children may benefit from drugs to treat specific symptoms such as attention disorder and seizures. Although autistic behaviors are the consequence of a static disorder of brain function, their character changes with maturation and appropriate intervention. Communication skills and sociability remain deficient but improve in all but the most severely affected children. Outcome is a function of both innate cognitive competence and the effectiveness of early intervention focused on the development of appropriate social skills and meaningful communication. Intelligent autistic adults may be educable, employable, and able to live independently, while more severely handicapped ones require a lifelong protected environment.

Autistic Disorder↗
PubMed1990

Computer assisted instruction for autistic children.

Since the beginning of 1980, Computer-Assisted-Instruction (CAI) has been used systematically in special education. The use of computers in the treatment of autistic children is highly controversial and emotional among parents and professionals. Fears of reinforcing autistic withdrawal are often mixed with insecurity and dislike of new technologies. On the other hand, positive effects of CAI on learning and behaviour are reported by parents and published as single case studies. The following paper relates perception, motivation, communication and behaviour--characteristics of autistic children to features of computer-assisted learning. Preliminary findings support the benefit of the use of computer-technology for the management of behaviour and learning of autistic children. In 12 autistics, video-taped evaluations showed higher enthusiasm ratings in computer-sessions than personal instruction sessions. Single case-studies demonstrated a positive influence of CAI on autistic children's behaviour-problems (e.g. avoidance of eye contact, echolalia) as well as improved spontaneous communication and better learning of academics.

Adolescent↗
PubMed1977

Deanol in Gilles de la Tourette Syndrome: a preliminary investigation.

On the basis of its pharmacologic action Deanol (dimethyl aminoethanol) was hypothesized to be of benefit in the Gilles de la Tourette Syndrome. In one case report the addition of Deanol to perphenazine did not result in an improvement of uncontrollable movements or involuntary speech utterances. Gilles de la Tourette Syndrome is a condition combining organic and psychogenic features existing in the interface between two etiologies. Classically the disease begins in childhood and is characterized by the appearance of sudden involuntary movements, involuntary speech utterances frequently consisting of curse words (coprolalia), and imitative phenomena such as echolalia and echopraxia. Neurotic symptomatology such as anxiety and obsessive thinking have also been reported. This condition is regarded neuropharmacologically as a dopaminergic state that responds to drugs with antidopaminergic activity e.g. the phenothiazines and butyrophenones. Deanol (dimethyl aminoethanol) is a putative cholinergic agonist and has reported effectiveness in conditions where there is a predominance of dopaminergic versus cholinergic activity, e.g. levodopa-induced dyskinesias, neuroleptic induced tardive dyskinesia, and Huntington's chorea. Because of its effectiveness in dopaminergic states it was hypothesized that Deanol could also be of benefit in the Gilles de la Tourette Syndrome.

Adult↗