Evidence of persisting cognitive impairment in a case series of patients with locked-in syndrome.

OBJECTIVE: Previous research on cognition and locked-in syndrome (LIS) is limited to single case reports, and usually reported normality of performance. Here, cognitive disorders in a group of LIS patients are investigated, using a specific test relying only on a yes/no response indicated by eye movements or verbally. METHODS: Nine patients with LIS resulting from brainstem stroke were compared with seven patients with frontal or frontotemporal lesions and 16 matching normal control subjects. The test comprised 19 subtests of perception (visual and auditory recognition), oral comprehension (identity, words and sentences), written comprehension (words and sentences), orientation (in time and place), immediate and delayed memories (verbal and visuo-spatial information), calculation and problem solving, and analysis of verbal logic. RESULTS: LIS patients showed significant difficulties in auditory recognition (associative level), oral comprehension of complex sentences, delayed visuospatial memory, mental calculation and problem solving. Patients with hemispheric lesions were more severely impaired. Single case analysis revealed that four LIS patients showed cognitive disorders in at least three subtests. These disorders were not related to a specific localisation of lesions. CONCLUSIONS: Moderate and selective cognitive disorders can be observed in LIS patients. Systematic assessment is needed, because of their possible consequences for communication and rehabilitation protocols.

Attempt to establish a classification of patients suffering from coma and admitted to a hospital structure for short or medium term treatment: medical aspects and costs of hospital care.

We dispose of a database, constituted between 1987 and 1993, containing medical and cost information concerning 515 patients suffering from coma and admitted, after a period of resuscitation, to a French hospital establishment--Etablissement Hélio-Marin of Berck-sur-Mer (EHMB)--for short and medium term treatment, between 1974 and 1986. From this base, which contains demographic and clinical data (age, sex, condition upon admission, duration of consciousness disorders, Glasgow Outcome Scale (GOS) upon discharge) we devised a hierarchical classification analysis following a factorial analysis of multiple correspondences, on 2 sets: a sample of 515 patients (all causes of coma being merged) and a sample of 266 patients suffering from brain injuries. Four groups were determined for each typology. These groups were first described on the basis of the variables used for their construction, and later by considering other available variables: origin of coma, duration of stay at EHMB, future evolution of patients and cost of treatment (cost of specific care, average daily cost, total cost of hospitalization). Thus, typical clinical situations were identified in each classification, depending on age of patient, origin of coma and condition upon admission. These situations led to extremely different treatment costs (ratio from 1 to 5 in the general typology and 1 to 2.85 in the classification of brain injuries.

Identification of new mutations in two phosphoglycerate kinase (PGK) variants expressing different clinical syndromes: PGK Créteil and PGK Amiens.

Phosphoglycerate kinase (PGK) deficiency is generally associated with chronic hemolytic anemia, although it can be accompanied by either mental retardation or muscular disease. Genomic DNAs of two PGK-deficient patients previously described in France were sequenced directly after polymerase chain reaction amplification. The PGK Créteil variant arises from a G-->A nucleotide interchange at position 1022 in cDNA (exon 9), resulting in amino acid substitution 314 Asp-->Asn in the C-terminal domain, which contains the nucleotide binding site. It is associated with rhabdomyolysis crises but not with hemolysis or mental retardation. In the other case, which is associated with chronic hemolytic anemia and mental retardation (PGK Amiens), an A-->T nucleotide interchange was found at position 571 in cDNA (exon 5); this leads to amino acid substitution 163 Asp-->Val in the N-terminal domain, which contains the catalytic site for phosphoglycerate binding. These results corroborate the kinetic data observed. In the two cases, the mutations are distinct from others previously reported and no significant relationship could be observed between the location of the amino acid substitution and its clinical consequences.

[Prognostic factors of post-traumatic vegetative states: 522 cases]. / Facteurs pronostics des états végétatifs post-traumatiques: 522 cas.

Long-term prognosis of post-traumatic vegetative state (VS) remains poorly defined. Three kinds of data have been collected for 522 patients in VS 1 month after head injury: pre-traumatic data; early data, collected during the first two weeks after trauma; late data, collected one month or more after trauma. Statistical relationships have been searched for, between these data and outcome one year after trauma, according to the Glasgow Outcome Scale. Some late data appear as essential prognostic factors of VS: threat blink; complications; ventricular dilatation; motor score (Glasgow Coma Score); spontaneous eye movements. Pretraumatic data then take place: age; previous deficiencies. Other data (especially early data) seem to have a weaker influence or outcome.

[Neurologic paraosteoarthropathy of the hand. Apropos of 2 cases]. / Paraostéoarthropathie nerveuse des mains. A propos de 2 observations.

The frequency of heterotopic ossification in neurological conditions averages 20-25% with variations according to the type of neurological disease and presence or absence of coma. The most common sites are the hips, knees, shoulders, and elbows. Involvement of the hands is an exceedingly rare event of which two instances are reported herein. In both patients evidence of inflammation developed four months after a deep, prolonged coma. Swanneck deformity of the fingers occurred in one case. Juxta-articular ossifications were seen on roentgenograms two months after the onset of symptoms. Both patients had heterotopic ossifications around the shoulders. Erythrocyte sedimentation rate and serum alkaline levels were elevated. Local injections effectively relieved pain in one patient.

[Problems of surveillance and nursing in chronic coma]. / Problèmes de surveillance et de nursing au cours des comas chroniques.

Impaired consciousness after severe brain damage is coma, the next step being vegetative state with a high mortality rate. Some vegetative patients may in fact improve, although they usually stay dependent. The entire attending and rehabilitation team looks for responses to simple orders, which mean return of consciousness, and watches the patient's articular state and potential complications. It is also necessary to maintain the patient's dignity at this stage of wakefulness without awareness, and to prevent neuro-orthopaedic and cutaneous complications by positions and mobilizations, in order to provide optimal conditions for a possible physical and/or mental recovery and to reduce the degree of dependence.

Obsessive-compulsive and other behavioural changes with bilateral basal ganglia lesions. A neuropsychological, magnetic resonance imaging and positron tomography study.

Eight patients are reported who shared the combination of bilateral basal ganglia lesions and a frontal lobe-like syndrome. The main features were inertia and loss of drive, with preservation of intellectual function. Some patients showed stereotyped activities with compulsive and obsessive behaviour which were sometimes highly elaborate in pattern. Extrapyramidal clinical signs were absent or mild. Brain damage, related to anoxic or toxic encephalopathy, was demonstrated by CT scans and MRI. The lesions appeared to be confined to the lentiform nuclei, particularly affecting the pallidum, although there was generalized brain atrophy in 2 cases. Positron emission tomography (PET) in 7 patients revealed hypometabolism of the prefrontal cortex relative to other parts of the brain. The PET studies suggest dysfunction of the prefrontal cortex as a result of damage to the lentiform nuclei. These clinical, anatomical and functional observations emphasize the role of the circuits linking the prefrontal associative cortex and some specific areas of the neostriatum, including the pallidum. The existence of distinct nonoverlapping circuits in the motor field or in the associative field can explain the fact that basal ganglia lesions may give rise to a clinical picture that is either purely motor, purely behavioural (as in some of our patients), or both. Similarities existed between some symptoms found in our patients and certain features of major psychiatric illnesses such as severe depression, catatonic schizophrenia, and obsessive-compulsive disorder. This raises the hypothesis that some aspects of these psychiatric disorders could be related to structural and physiological disturbances in the systems linking the frontal associative cortex and the basal ganglia.

Chronic vegetative state after severe head injury: clinical study; electrophysiological investigations and CT scan in 15 cases.

Fifteen cases of chronic vegetative state (CVS), following severe head injury and lasting for two years or more, are reported. Vegetative state, in most instances after a period of coma, consists of a return of wakefulness accompanied by an apparent total lack of higher mental activity. A protracted period of vegetative state has been chosen to ensure that the possibility of further recovery could virtually be excluded. The term of CVS could therefore be reasonably used to designate these cases. Moreover, cerebral lesions were then thought to be the same as in neuropathological studies. Severe head injury, responsible for CVS, initially affected adults in 11 cases and children in four cases. The range of duration of the vegetative state was 2 to 14 years, with a mean of five years. The data of clinical study and electrophysiological investigations (EEG, brain stem auditory evoked potentials, somatosensory evoked potentials) are reported. A CT scan was carried out in each case to study the impairment of cerebral hemispheres and brain stem, with particular attention to the ventricular size. The results confirm that in the CVS, lesions affect mainly the hemispheres, while brain stem functions are mainly preserved. Vegetative State (VS) is the term proposed by Jennett and Plum (1972) to describe the condition that sometimes emerges after a period of coma, after a severe head injury (SHI). This condition consists of a return of wakefulness accompanied by an apparent total lack of higher mental activity. A practical definition of this state characterised by wakefulness without responsiveness is that the eyes open spontaneously and/or in response to verbal stimuli. Sleep-wake cycles exist. The patients can neither obey simple orders nor locate painful stimuli. They utter no comprehensible words. Blood pressure and breathing remain steady. It is much more difficult to specify exactly how long such a state must persist before it can be confidently declared permanent. Persistent vegetative state, or chronic vegetative state (CVS) is one of the five categories of the Glasgow Outcome Scale (Jennett and Bond, 1975). Bricolo et al (1980) think that the term CVS should never be applied before completion of the first year after the onset of traumatic coma. It actually seems possible to exclude the possibility of any further recovery after unresponsiveness for one year. The term CVS may then be rightfully used to denote this condition. For such protracted periods of VS, we have tried to state in a retrospective study the clinical and electroencephalographic (EEG) course.(ABSTRACT TRUNCATED AT 400 WORDS)

Cholinergic-dependent cognitive deficits in Parkinson's disease.

In a double-blind cross-over study, the effects of a subthreshold dose of scopolamine (0.25 mg) on memory were compared in 32 control subjects and 32 parkinsonian patients who were without any sign of intellectual and mnemic impairment. Although the scores of the controls in the memory test battery showed no deterioration after the administration of scopolamine, the same dose resulted in significantly reduced memory performance in parkinsonian patients in two tests which involved the recognition of meaningless drawings. The selective vulnerability of parkinsonian subjects without cognitive impairment to a subthreshold dose of scopolamine suggests the existence of an underlying alteration of central cholinergic transmission. The neuropsychological findings in our study agree with postmortem biochemical data, which showed decreased cortical choline acetyltransferase activity in all parkinsonian patients, suggesting the existence of neuronal compensation in parkinsonian patients who are without cognitive impairment.

[Meningo-myelitis with a favorable course after measles vaccination]. / Méningo-myélite d'évolution favorable après vaccination antirubéolique.

A 27 year-old woman complained of symptoms and signs due to a meningo-myelitis, 5 days after anti-rubella vaccination. Among the earliest symptoms she experienced pain and sensory deficit in the inoculated arm. The disease worsened until the 15th day, then improved spontaneously.

[Benign intracranial hypertension (pseudotumor cerebri). 12 cases]. / Hypertension intracrânienne bénigne (pseudo tumor cerebri). Douze cas.

Twelve cases of benign intracranial hypertension (BIH) are reported, there being a higher proportion of women patients and a greater frequency of those in the 3rd decade of life. Symptoms were principally headache (11 patients) and/or transient or permanent visual disorders (10 patients). Bilateral papillary edema was often associated with diminished visual acuity (5 cases) and/or amputation of the visual field (5 cases). Cerebral venous occlusion was detected in 3 patients and an infective focus in 7 cases. Three patients were receiving some treatment but none of the women was taking oral contraceptives. Two were pregnant. The CSF was abnormal in 5 of the 7 patients (mixed cell reaction and red blood cells: 1 case; pleiocytosis: 3 cases; raised protein levels: 1 case), and CT scans were normal except for reduced ventricular system size in 9 patients. Follow-up for a mean of 1 year showed total disappearance of symptoms and signs in 4 cases. Papillary edema had not totally regressed at the last ophthalmological examinations performed on 5 patients, and visual sequelae (visual acuity and/or visual field anomalies) were present in 5 cases. Two patients relapsed. The conventional acceptance of the benign nature of BIH must be tempered by the possible onset of possible serious visual disorders. Two factors appear to be determinant: the presence of severe visual disorders initially and/or the existence of arterial hypertension. Various physiopathogenic hypotheses have been evoked to explain BIH. The essential mechanism appears to be an anomaly of absorption of CSF, either from increased pressure in the dural venous sinuses or lesions of the arachnoid villi, but the role of cerebral edema and/or increased cerebral blood volume cannot be excluded. Incomplete understanding of physiopathogenic mechanisms account for the numerous treatments proposed and for some confusion in their indications.