Depression, anxiety and non-motor symptoms on initiation of intrajejunal levodopa/carbidopa therapy.

In Parkinson's disease (PD), clinical observations and some studies suggest that depression and anxiety are linked to motor fluctuations. We studied prospectively 10 patients with advanced PD just before initiation of intrajejunal levodopa/carbidopa therapy, and after 1 and 3 months of regular treatment. Motor symptoms, motor fluctuations, non-motor symptoms, quality of sleep, symptoms of depression and anxiety were evaluated with the appropriate scales. As expected, motor symptoms and motor fluctuations improved considerably. Non-motor symptoms, quality of sleep and depression also improved significantly. However, anxiety score remained unchanged during the study. Our data in a small numbers of patients indicate that all aspects of mental and psychic symptoms are not alleviated within a short period of reduction of motor fluctuations.

Painful arm and moving fingers: clinical features of four new cases.

The syndrome of painful arm and moving fingers associates pain in one arm or hand with involuntary movement of one or several fingers. In the four cases described, an association between a central and a peripheral nervous system lesion is demonstrated or suspected. Treatment of the condition is disappointing.

[The neurology department]. / Le Service de Neurologie.

The department of neurology is devoted to the diagnosis and medical treatment of organic diseases of central nervous system (brain and spinal cord) and peripheral nervous system (peripheral nerves and muscles). Basic and clinical research in neuroscience constitute an essential activity of the department that defines its academic character. Over the years, the department of neurology has evolved from providing general neurology services to a multifaceted unit that has developed the several subspecialties of clinical neuroscience. Main research areas have included neurooncology, neurophysiology, neuropsychology, cerebrovascular diseases, childhood epilepsy and conditions affecting the psychomotor development of children. Neurogenetics is a recent addition to the areas of the interest of the department; research in neurogenetics includes basic investigations as well as clinical studies and focuses on inherited ataxias and genetic epilepsies.

[Encephalomyelopathy associated with monoclonal gammapathy]. / Encéphalomyélopathie associée à une gammapathie monoclonale.

We describe the case of a patient with a particular form of presumably immune-mediated encephalomyelitis associated with a monoclonal cold agglutin gammapathy. Systematic autopsy showed predominantly demyelinating lesions of the brain and spinal cord. The lesions were assumed to be the immune-mediated consequences of the underlying hematologic condition. Similarity with certain paraneoplastic syndromes is underlined.

Frontotemporal dementia: a clinical-pathological study.

We report a 44-year-old female patient without any familial history of dementia presenting with increasing disturbances in behaviour and language followed by a progressive cognitive deterioration. Neuropsychological evaluation revealed a significant impairment on frontal lobe tests. A brain PET scan disclosed a severe frontal hypometabolism. The tentative diagnosis of frontotemporal dementia was made. Her condition rapidly worsened and she died 2 years after the beginning of her disease. Gross examination of the brain showed a selective symmetrical atrophy of both frontal and anterior part of the temporal lobes. Microscopical examination revealed severe neuronal loss in the frontal and anterior temporal cortex associated with gliosis and microvascular spongiosis in the superficial cortical layers in the absence of any specific neuronal or glial inclusions. These neuropathological findings were consistent with the diagnosis of dementia lacking distinctive histology. We discuss the nosology of the frontotemporal dementias, the diagnostic value of PET scan, the recent genetical developments which strongly support the pathogenic role of tau and we emphasize the importance of immunohistochemical examination for a definite neuropathological diagnosis.

[Dopaminergic agonists in the treatment of Parkinson's disease]. / Les agonistes dopaminergiques dans le traitement de la maladie de Parkinson.

After levodopa, dopaminergic agonists are the most powerful agents in idiopathic Parkinson's disease treatment. Used in monotherapy or rather in early combination with levodopa, they allow a dramatic reduction of long-term motor side effects of the latter: onset and peak-dose dyskinesias, early morning dystonias. Their gastro-intestinal (nauseas) and moreover psychiatric (confusion and hallucinations) side effects limit their use, notably in geriatric populations. Superiority of so-called "second generation" agonists (ropinirole, pramipexole) on "first generation" agonists (bromocriptine, pergolide) remains to be proved.

[Surgical treatment of Parkinson disease: indications and limitations]. / Traitement chirurgical de la maladie de Parkinson: indications et limites.

Thalamotomy, a surgical lesion of the ventro-intermediate nucleus of the thalamus, is a well known surgical treatment of tremor in Parkinson's disease. Over the last years, new surgical therapies had been developed. These therapies, instead of making a lesion in the brain, consist in placing electrodes in specific areas of the brain and to inhibit neuronal function by electrical stimulation. Electrical stimulation of the subthalamic nucleus or of the pallidum are effective to treat motor symptoms of Parkinson's disease. The procedure can be done bilaterally, contrary to thalamotomy. A short overview of the indications and contra-indications of this kind of therapy is given.

Audiogenic startle reflex in acute hemiplegia.

The generators of the audiogenic startle reflex (ASR) are located in the bulbopontine reticular formation. We studied the influence of acute vascular supratentorial lesions on ASR. Ten patients with hemiplegia due to hemispheric cerebral infarct were studied within 5 days of stroke onset. ASR and magnetic cortical stimulation were performed the same day. A muscle response to magnetic stimulation was not elicited over the plegic side in any patient. In four of seven patients, ASR was enhanced over the plegic side. We suggest that enhanced ASR is due to the loss of a predominantly inhibitory hemispheric drive on ASR generators.

Central nervous system lesions associated with Crohn's disease.

Central nervous system vasculitis is an exceptional extraintestinal manifestation of Crohn's disease. Reported here are 2 cases, highlighting the difficulty of differential-diagnosis with multiple sclerosis and stressing the importance of early immuno-suppressive therapy.

Effects of vigabatrin on motor potentials evoked with magnetic stimulation.

We studied the effect of an acute loading dose of vigabatrin on threshold of motor responses and duration of silent period elicited with cortical magnetic stimulation in normal subjects. In contrast to phenytoin, vigabatrin does not increase the motor threshold of first dorsal interosseus muscle. We also show that, although vigabatrin increases GABA concentrations in the central nervous system, duration of silent period studied at various stimulus intensities is not modified after vigabatrin administration.

Chronic intrathecal baclofen in severely disabling spasticity: selection, clinical assessment and long-term benefit.

Flexor and extensor spasms associated with severe spasticity frequently cause pain and suffering in neurologically impaired patients, and greatly interfere with comfort and activities. When high doses of oral medications are necessary to keep the symptoms under control and are poorly tolerated, the long-term spinal-selective intrathecal infusion of baclofen by means of implanted drug pump and catheter is a safe, efficient and reversible alternative to destructive surgical procedures. Between September 1991 and March 1995, intrathecal baclofen was infused in 18 selected patients out of a series of 42 severely disabled spastic cases. We report here our preliminary experience with the criteria of selection, the initial intrathecal bolus test and the long-term benefit of the selected patients. Our results confirm the dramatic immediate and long-term benefit reported in other series. After a period of treatment of 1 to 42 months, 13 patients had a complete disappearance of their spastic symptoms without any oral treatment, one patient kept unchanged clonus despite the use of low-dose oral treatment and another one a severe, not improved dysuria although in both of them hypertonia and spasms were abolished. Finally, 2 patients had important joint stiffness slightly impairing the benefit from the treatment. None of the 18 patients had central side-effects related to baclofen. With time, a slight increase in daily dose (inferior to 10%) was necessary in most patients.

Abnormal motor evoked responses to transcranial magnetic stimulation in focal dystonia.

We evaluated motor responses evoked after magnetic cortical stimulation in dystonia, emphasizing the relationship between resting and facilitation state. We studied 15 normal controls (mean age, 37.9 years; range, 23 to 63) and 13 dystonic patients (mean age, 43.4 years; range, 20 to 56). Surface electrodes were placed over the right first dorsal interosseous muscle to measure motor evoked potentials and inhibitory silent periods obtained with magnetic stimulation. The amplitude ratio of motor evoked potentials measured during facilitation and at rest with low-intensity magnetic stimulation was significantly higher in dystonic patients (15.09) when compared with normal subjects (5.43; p = 0.04). The ratio of duration of silent periods evoked with 120% motor threshold (MT) and MT + 25% magnetic stimulus intensity was significantly higher in dystonic patients (78.4%) when compared with normal subjects (69.7%; p = 0.04). We conclude that with low-intensity magnetic stimulation the relationship between amplitudes of motor potentials evoked at rest and during facilitation, as well as the responses of pathways that mediate silent periods, are disturbed in focal dystonia.

Effects of diphenylhydantoin on motor potentials evoked with magnetic stimulation.

We studied the effect of an acute loading dose of diphenylhydantoin (DPH) on motor responses elicited with cortical magnetic stimulation in normal subjects. DPH increased significantly the motor threshold activation of ADM, APB, FDI and biceps. The motor threshold increase was of greater magnitude for the proximal muscle. Spinal soleus alpha-motoneuron pool excitability assessed by H-reflex was increased significantly suggesting that the motor threshold increase is related to a supraspinal effect of the drug. Our study demonstrates that the motor threshold increase observed after DPH administration occurs not only in epileptic patients but also in normal subjects.

Somatosensory central conduction time after sural and tibial nerve stimulation.

We recorded spinal and cortical somatosensory evoked potentials (SEPs) after sural and tibial nerve stimulation at the ankle in 34 normal subjects. Spinal SEPs were reproducible with sural nerve stimulation in only 65% of normal subjects. The spinal amplitudes were significantly smaller after sural nerve stimulation. Central conduction time (CCT) was significantly shorter when measured from onset instead of peak latencies. There was a significant difference between CCT with tibial nerve and sural nerve stimulation. Our results are consistent with the idea that CCT measures from onset and peak latencies do reflect the travel of the afferent volley in different spinal fiber populations.

Spinal and brain-stem SEPs and H reflex during enflurane anesthesia.

Whereas cortical SEPs are altered by halogenated anesthetics, spinal and subcortical SEPs are thought to be hardly affected. In this study the spinal N13 potential (recorded with anterior neck reference) showed a significant delay with enflurane anesthesia. The P13 and P14 far-field potentials, however, remained unchanged. Our results indicate that oligosynaptic as well as polysynaptic pathways are influenced by halogenated anesthetics and that enflurane has different effects on spinal gray matter and cuneate synapses. Our data also demonstrate that earlobe reference recordings are not adequate to measure pharmacologic effects on subcortical SEPs.

Dissociation of P13-P14 far-field potentials: clinical and MRI correlation.

P13 and P14 far-field potentials are recorded over the scalp with median nerve stimulation when non-cephalic reference is used to measure somatosensory evoked potentials. The dissociation of these 2 potentials is exceptional. Only 2 cases subsequent to pontine lesions have been described hitherto. We report the case of a 31-year-old woman with a low grade glioma located at the spino-medullary junction who presented a P13-P14 far-field dissociation. This case fully supports the independent nature of the P13 and P14 potential generators.

Topographic analysis of the effects of isoflurane anesthesia on SEP.

Isoflurane anesthesia induces a striking increase in the P22 potential recorded over the precentral scalp whereas the amplitude of the N20 is reduced. It is not known whether the increased "P22" enhanced by isoflurane arises from the same generator as the small precentral P22 potential recorded in the normal awake subject. Multi-channel recordings of SEP before and during isoflurane anesthesia were performed in 13 normal subjects. Isopotential topographic maps showed that isoflurane did not change the distribution of the precentral "P22" despite its clear amplitude increase. Our data confirm that isoflurane enhances the precentral P22 and that the enhanced "P22" arises from the same generator as the P22 recorded before isoflurane anesthesia.