Anthropological neurology: symptoms and their meanings according to Joseph Prick (1909-1978).

This article describes the life and work of the Dutch neurologist Joseph Prick (1909-1978) and his idea of an anthropological neurology. According to Prick, neurological symptoms should not only be explained from an underlying physico-chemical substrate but also be regarded as meaningful. We present an outline of the historical and philosophical context of his ideas with a focus on the theory of the human body by the French philosopher Maurice Merleau-Ponty (1908-1961) and the concept of anthropology-based medicine developed by Frederik Buytendijk (1887-1974). We give an overview of anthropological neurology as a clinical practice and finally we discuss the value of Prick's approach for clinical neurology today.

Acute severe memory impairment as a presenting symptom of multiple sclerosis: a clinical case study with 3D double inversion recovery MR imaging.

We present the case of a 21-year-old woman with acute memory impairment. It was initially not recognized as organic in nature. Repeated neuropsychological testing confirmed severely disturbed encoding and recall. Three-dimensional double inversion recovery (3D-DIR) MRI confirmed hippocampal and cortical lesions. Further clinical symptoms, visual evoked potential and MRI suggested a diagnosis of multiple sclerosis (MS). The amnestic syndrome has remained for 5 years. This case illustrates that MS can present with acute cortical symptoms which can be difficult to recognize. The hippocampus can be acutely affected and DIR MRI imaging can help to demonstrate cortical lesions that explain the clinical picture.

[Explaining symptoms: body-object and body-subject]. / Het verklaren van symptomen: lichaam-object en lichaam-subject.

BACKGROUND: Medically unexplained somatic symptoms present a real challenge to modem medicine. They occur frequently and are often chronic. AIM: To discuss the physician's approach to these symptoms. METHOD: We focus on the views of the human body put forward by the French philosopher Merleau-Ponty. His view is illustrated with the help of a case study concerning a patient suffering from borderline personality disorder and complex regional pain syndrome. RESULTS: Merleau-Ponty draws a distinction between body-subject and body-object. By body-object he means the body as determined and predicted by medical science. By body-subject he means the body as we experience it, even before we are aware of it. It is our pre-reflexive body that gives meaning to the world around us. CONCLUSION: We argue that the physician should regard the body not only as an object, but also as a subject, thereby giving renewed attention to the significance of symptoms as an expression of inner experience. In this way symptoms that cannot be explained on a purely physico-chemical level will become more meaningful.

[A meningioma resulting in drastic changes in behavior and life course]. / Een meningeoom als oorzaak van ingrijpende veranderingen in gedrag en levensloop.

Three patients, a 59-year-old woman, a 51-year-old man and a 66-year-old woman presented at a neurological or neurosurgical outpatient clinic after their lives had been drastically affected by behavioural changes. Neither the patients nor their relatives or friends were aware that the behavioural changes may be caused by a medical problem. The cause, a large meningioma, became obvious only after physical symptoms occurred a few years later. The patients recovered after surgery and regretted having made certain decisions with far-reaching repercussions for their lives and families. Behavioural disturbances are seldom due to slowly growing brain tumours, but consultation with a neurologist is recommended if there is any doubt regarding the possibility of somatic causes.

Gilles de la Tourette's syndrome with and without obsessive-compulsive disorder compared with obsessive-compulsive disorder without tics: which symptoms discriminate?

Stereotyped repetitive behaviors occur in Gilles de la Tourette's Syndrome (GTS) and obsessive-compulsive disorder (OCD). The present study was undertaken to compare the distribution of obsessive-compulsive and Tourette-related impulsive behaviors in GTS with (+) OCD, GTS without (-) OCD, tic-free OCD, and control subjects. Fourteen GTS + OCD, 18 GTS-OCD, 21 OCD-tic, and 29 control subjects were evaluated using a semistructured interview designed to assess GTS and OCD-related repetitive behaviors. Each reported item was evaluated on the presence of anxiety and goal-directedness. This information was subsequently used to define whether the repetitive behavior was an (anxiety-related) obsession or compulsion, or a (non-anxiety-related) OC-like behavior, impulsion. GTS + OCD subjects reported more overall Tourette-related impulsions than OCD-tic subjects, i.e., more mental play, echophenomena, and touching behaviors but similar frequencies of typical obsessive-compulsive behaviors. Further, GTS + OCD subjects exhibited more overall repetitive behaviors than GTS-OCD subjects, i.e., more Tourette-related impulsions as well as more obsessive-compulsive behaviors. The distribution of symptoms is similar in GTS with and without OCD, and differs from tic-free OCD. These differences suggest that GTS with OCD constitutes a form of GTS, not of OCD, although the possibility that GTS + OCD patients constitute a subgroup distinct from GTS and from OCD can not be excluded by this phenomenological study. Specific non-anxiety-related impulsions seem to discriminate between GTS and OCD-tic individuals. These impulsions possibly reflect differences in underlying mechanisms between GTS and OCD-tics.

Repetitive behaviors in Tourette's syndrome and OCD with and without tics: what are the differences?

Gilles de la Tourette Syndrome (GTS) and obsessive-compulsive disorder (OCD) share obsessive-compulsive phenomena. The aims of this study were to compare the OC symptom distribution between GTS and OCD and to investigate whether a subdivision of these phenomena into obsessions, compulsions and 'impulsions' is useful in distinguishing GTS and OCD patients. Thirty-two GTS, 31 OCD (10 with tics, 21 without tics) and 29 control subjects were studied using the Leiden repetitive behaviors semi-structured interview to assess GTS as well as OCD-related behaviors. Each reported repetitive thought or action was evaluated on the presence of anxiety and on goal-directedness. This information was used to define whether the behavior was an obsession, compulsion, or 'impulsion'. Both the GTS and OCD study groups showed higher scores than control subjects on rating scales measuring depression, OC behavior and anxiety. In GTS, Y-BOCS severity scores and trait anxiety were lower than in the OCD groups. Furthermore, GTS patients differed from OCD patients in the distribution of symptoms. Aggressive repetitive thoughts, contamination worries and washing behaviors were reported more frequently by tic-free OCD, while mental play, echophenomena, touching and (self)-injurious behaviors were reported more frequently by GTS. OCD individuals with tics were intermediate, but closer to tic-free OCD. GTS individuals reported significantly more 'impulsions' and fewer obsessions and compulsions than OCD individuals with and without tics. Factor analysis revealed three factors accounting for 44% of the variance, resulting in an 'impulsive' factor related to GTS, a 'compulsive' factor related to OCD and an 'obsessive' factor related to tic-free OCD. In conclusion, OCD individuals reported more anxiety and goal-directedness associated with their behaviors than did GTS subjects. The distinction between obsessions, compulsions and impulsions is of importance in identifying Tourette-related vs. non-Tourette-related repetitions.

The relationship between types and severity of repetitive behaviors in Gilles de la Tourette's disorder and obsessive-compulsive disorder.

BACKGROUND: This study investigated which categories of obsessive-compulsive and Tourette-related behaviors in Gilles de la Tourette's disorder and obsessive-compulsive disorder (OCD) without tics are experienced as most severe across the study groups and what the differences are in symptom distribution between the study groups. METHOD: Fourteen subjects with both Tourette's disorder and OCD, 18 subjects with Tourette's only, 21 subjects with OCD (no tics), and 29 control subjects were studied using a semistructured interview designed to equally assess Tourette- and OCD-related behaviors according to DSM-III-R criteria. Each reported repetitive behavior was evaluated on the presence of anxiety and on goal-directedness. Anxiety-related items were categorized as obsessions or compulsions and non-anxiety-related items as impulsions. Severity of each reported item was assessed with respect to time per day consumed and amount of distress and interference induced by the item. Following these criteria, each reported item was classified as a symptom, a subthreshold symptom, or just as being present. RESULTS: Across the study groups, obsessions were experienced as more severe than (Tourette-related) impulsions and compulsions. Within the study groups, patients with both Tourette's disorder and OCD reported more symptomatic Tourette-related impulsions, such as mental play, echophenomena, and impulsive or self-injurious behaviors; less overall symptomatic obsessions; and less symptomatic washing than patients with OCD (no tics). The differences among individuals with Tourette's with or without OCD reflected differences in symptom severity rather than differences in symptom distribution. CONCLUSION: Obsessions are more time consuming, distressing, and interfering than compulsions and impulsions. Furthermore, the symptomatic repetitive behaviors were distributed differently among patients with both Tourette's disorder and OCD and patients with OCD (no tics). Patients with Tourette's and OCD are phenomenologically more similar to Tourette's than to OCD. These differences possibly represent differences in underlying pathophysiology between Tourette's and tic-free OCD.

Non-linear analysis of the electroencephalogram in Creutzfeldt-Jakob disease.

Creutzfeldt-Jakob disease is a rare, neurological, dementing disorder characterised by periodic sharp waves in the electroencephalogram (EEG). Non-linear analysis of these EEG changes may provide insight into the abnormal dynamics of cortical neural networks in this disorder. Babloyantz et al. have suggested that the periodic sharp waves reflect low-dimensional chaotic dynamics in the brain. In the present study this hypothesis was re-examined using newly developed techniques for non-linear time series analysis. We analysed the EEG of a patient with autopsy-proven Creutzfeldt-Jakob disease using the method of non-linear forecasting as introduced by Sugihara and May, and we tested for non-linearity with amplitude-adjusted, phase-randomised surrogate data. Two epochs with generalised periodic sharp waves showed clear evidence for non-linearity. These epochs could be predicted better and further ahead in time than most of the irregular background activity. Testing against cycle-randomised surrogate data and close inspection of the periodograms showed that the non-linearity of the periodic sharp waves may be better explained by quasi-periodicity than by low-dimensional chaos. The EEG further displayed at least one example of a sudden, large qualitative change in the dynamics, highly suggestive of a bifurcation. The presence of quasi-periodicity and bifurcations strongly argues for the use of a non-linear model to describe the EEG in Creutzfeldt-Jakob disease.

[Asystole due to complex partial epilepsy]. / Asystolie door complexe partiële epilepsie.

A 56-year-old man was seen in the Emergency Room because of sudden and transient loss of consciousness. An asystole was diagnosed with 10 S duration after which a pacemaker was implanted. The patient had experienced such collapses before, preceded by odd smell sensations. After the implantation of the pacemaker, the patient did not collapse again but still experienced attacks of the same smell sensations combined with a strange feeling in his stomach. An EEG, recorded during such an attack, showed epileptic activity predominantly over the right hemisphere while at the same moment a pacemaker rhythm was observed. After the epileptic activity had stopped, normal sinus rhythm reappeared. On anticonvulsant therapy the attacks disappeared completely.

Use of non-linear EEG measures to characterize EEG changes during mental activity.

Non-linear EEG analysis provides a possibility for studying the dynamical changes in cortical networks related to mental activity. In this study the correlation dimension D2 was used to study local changes in complexity, and the mutual dimension Dm was used to assess changes in the dynamical coupling between different brain areas. EEGs were recorded in 25 healthy subjects under three conditions: (1) eyes closed, (2) eyes open, and (3) mental arithmetic with eyes closed (serial subtraction of 7s from 1000). In the eyes-closed condition, D2 was lower at parieto-occipital sites. D2 increased during the eye-open and arithmetic conditions. Contrary to the D2, the Dm showed no regional differences in the eyes closed condition. A clear increase in Dm was seen during eyes open and arithmetic. We conclude that both the correlation dimension and the mutual dimension are very sensitive to EEG changes during simple visual information processing and during mental arithmetic. However, these measures seem to be relatively non-specific, and correlate only weakly with performance on the arithmetic task.

Altered attentional processing of background stimuli in Gilles de la Tourette syndrome: a study in auditory event-related potentials evoked in an oddball paradigm.

In 24 adults and 29 children both with Gilles de la Tourette syndrome and in 24 control adults and 17 control children we studied auditory event-related potentials (ERP's) evoked in 2 different oddball paradigms: a passive listening paradigm (PLP) in which subjects had only to attend tones (260 standards, 40 deviants), followed by an active discrimination paradigm (ADP) in which subjects had to press a microswitch in response to the deviant tones. In the adult Tourette patients the PLP disclosed between 200-300 msec at all derivations (Fz, Cz, Pz, C3, C4, P3, P4) a significantly more negative activity than in the controls. In the Tourette children such a result was only found at Fz. In the ADP the differences between both adult groups disappeared, but the curves of the control children became now significantly more positive than those of the Tourette ones except at C3 and P3. Between 200-300 msec the curves of all 4 groups were significantly less negative in the ADP than in the PLP. This decrease in negativity was significantly larger in the adult Tourette patients than in the adult controls, but in the children groups the reverse occurred. It is hypothesized that the differences in amplitude between patients and controls and between both paradigms are due to differences in amplitude of an endogenous negative component overlapping the exogenous N100-N200 standard curve. Based on this hypothesis the results could suggest that one of the problems in Tourette syndrome is an increased attention to non-relevant stimuli.

EEG changes during mental activation.

Desynchronization of the EEG in relation to mental activity can be demonstrated through various means. The acceleration spectrum entropy (ASE) is a method that quantifies the desynchronization of the EEG. It is a measure of the randomness or irregularity of a time series and ranges from 0 (straight line) to 100 (discrete random signal). The ASE was calculated for 20 EEGs under four conditions: "eyes closed," "eyes open," "eyes closed and mental arithmetic" and "eyes open and mental arithmetic." There was a significant increase between the conditions "eyes closed" and "eyes open" and a significant increase between the conditions "eyes closed" and "eyes closed and mental arithmetic." During eye opening the largest increase was seen in the occipital areas. Mental arithmetic showed an asymmetrical increase, namely in the left temporo-occipital region and in the right centro-temporal region. It is suggested that the ASE can be used to study cortical activation due to mental activity.

Mental play in Gilles de la Tourette's syndrome and obsessive-compulsive disorder.

A new phenomenon, found only in Gilles de la Tourette (GTS) patients, and which we have called 'mental play', is described. It was compared with the phenomenon of counting, which occurred in both GTS and obsessive-compulsive patients. In the GTS patients both mental play and counting were best characterised as playful impulsions. In contrast to the GTS patients, the counting of the obsessive-compulsive patients was in line with their obsessive-compulsive behaviour. These findings suggest that repetitive symptoms in GTS patients, even when they share superficial similarities with obsessive-compulsive symptoms, should not be diagnosed automatically as obsessive-compulsive.

Carbamyl phosphate synthetase-1 deficiency discovered after valproic acid-induced coma.

Valproic acid induced coma is presented in an adult patient without a history of metabolic disease. Liver biopsy revealed a reduction in activity of carbamyl phosphate synthetase-I, an enzyme obligated for transformation of ammonia to urea in the urea cycle. After recovery CT scan follow-up showed marked cerebral atrophy which did not exist prior to the state of coma. Risk factors are discussed.

Transcranial magnetic stimulation in peripheral facial nerve palsy of central origin.

We describe a 24-year-old woman with a peripheral facial nerve palsy associated with one-and-a-half syndrome due to multiple sclerosis. Transcranial magnetic stimulation located the facial nerve dysfunction in the brainstem.