[Peripheral neuropathies, from diagnosis to treatment, review of the literature and lessons from the local experience]. / Les neuropathies périphériques, du diagnostic au traitement, revue de la littérature et enseignements de l'expérience locale.

Peripheral neuropathy implies damages to neurons belonging to the peripheral nervous system which includes cranial nerves, spinal nerves' roots, spinal ganglia, nerve trunks and their divisions, and, the autonomic nervous system. Peripheral neuropathies are frequent in the general population (prevalence: 2,4%). We present a review of the recent literature and highlight diagnostic approaches for certain types of neuropathies particularly the most frequent ones or those requiring peculiar attention in first-line medicine. We also present epidemiologic data and data related to sural nerve biopsies from our centre. The determination of the location and the topography of the affected sites, integrated into the global context of the patient, is essential to provide an etiologic diagnosis. The median nerve compression within the carpal tunnel and polyneuropathies are the most frequent forms of peripheral neuropathies. More than one hundred causes of polyneuropathies are described and they are divided into acquired, genetically determined and idiopathic. We highlight a largely adopted diagnostic strategy concerning polyneuropathies and describe the Guillain-Barre syndrome, the alcohol-related polyneuropathy and the controversies about the benefit of the B vitamin therapy and its dangers. At the Hôpital Erasme, since 2008, more than 1372 patients with peripheral neuropathy were identified. Results of sural nerve biopsies performed in seventeen of them do not largely differ from those of other centres of expertise. We conclude that the diagnosis of peripheral neuropathy usually requires the expertise of a neurologist, but, first line caregivers must be able to recognize and refer patient when needed.

[Restless legs syndrome. Recommended drug management in 2007]. / Syndrome des jambes sans repos. Traitements recommandés en 2007.

Restless legs syndrome is a common sensorimotor disorder occurring in approximately 10% of the general population. The improved knowledge of the pathophysiology of the syndrome, the increased number of molecules which proved their efficacy for symptoms control in well conducted studies allow clinicians to propose a therapeutic approach tailored for the individual patient with a good chance of positive response.

Chronic inflammatory demyelinating polyneuropathy in a diabetic patient: deterioration after intravenous immunoglobulins treatment and favorable response to steroid treatment.

The authors report the case of a 54-year old type-2 diabetic female patient with a Chronic Inflammatory Demyelinating Polyneuropathy (CIDP). She progressively developed distal hypoesthesia and tetraparesis. She deteriorated after two courses of intravenous immunoglobulins (IVIG) administration and became rapidly wheelchair bound. After one month of steroid treatment, the patient was walking alone. This case raises the question whether IVIG is to be considered as first line treatment for diabetes associated CIDP.

[Cerebral functions in brain-damaged patients. What is meant by coma, vegetative state, minimally conscious state, locked-in syndrome and brain death?]. / Zerebrale Funktionen bei hirngeschädigten Patienten. Was bedeuten Koma, "vegetative state", "minimally conscious state", "Locked-in-Syndrom" und Hirntod?

Comatose, vegetative, minimally conscious or locked-in patients represent a problem in terms of diagnosis, prognosis, treatment and everyday management at the intensive care unit. The evaluation of possible cognitive functions in these patients is difficult because voluntary movements may be very small, inconsistent and easily exhausted. Functional neuroimaging cannot replace the clinical assessment of patients with altered states of consciousness. Nevertheless, it can describe objectively how deviant from normal the cerebral activity is and its regional distribution at rest and under various conditions of stimulation. The quantification of brain activity differentiates patients who sometimes only differ by a brief and incomplete blink of an eye. In the present paper, we will first try to define consciousness as it can be assessed at the patient's bedside. We then review the major clinical entities of altered states of consciousness encountered in the intensive care unit. Finally, we discuss the functional neuroanatomy of these conditions as assessed by positron emission tomography (PET) scanning.

Antiphospholipid antibodies and ischemic neuropathy following cardiac surgery.

Sciatic nerve palsy is an uncommon complication of cardiac surgery and is thought to be induced by a combination of reduced femoral artery blood flow, small vessel vascular disease or prolonged hypoxia. We here describe a new case which is the first described with transient elevation of antiphospholipid antibodies. Although transient elevation of lupus coagulation inhibitor is known to occur frequently in patients treated in an intensive care unit, there are very few data about the possible role of antiphospholipid antibodies in the generation of ischemic neuropathies. We can not prove that the ischemic neuropathy in our case has been favored by the presence of lupus coagulation inhibitor and antiphospholipid antibodies as the occurrence of the symptoms seemed to precede the transient elevation of lupus coagulation inhibitor. This case suggests that antiphospholipid antibodies and lupus coagulation inhibitor should be included in the work up of patients who present nerve damage after cardiac surgery but further studies are needed to ascertain this association.

[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.

Hepatic myelopathy after splenorenal shunting: report of one case and review of the literature.

Hepatic myelopathy is a rare complication of cirrhosis, usually associated with surgical or spontaneous porto-systemic shunts. Its pathophysiology is unknown. It is characterized by a motor involvement of the lower limbs without clinical sensory abnormality, leading to spastic paraparesis. These neurological features are related to a symmetric loss of myelin in the lateral corticospinal tracts. Usefulness of liver transplantation in this setting is not yet determined. We describe here the case of a 29-year-old male who presented with progressive spastic paraparesis of the lower limbs 3 years after a spleno-renal shunt.

Encephalomyelitis and bilateral optic perineuritis after influenza vaccination.

We report the case of one patient suffering from headache, urinary retention, bilateral optic disc swelling and a mild bilateral visual defect after influenza vaccination. The presumptive diagnosis was encephalomyelitis with bilateral optic perineuritis caused by influenza vaccination. We stress on the interest to search for this aetiology in unexplained optic neuropathy.

Electroencephalographic abnormalities in borderline personality disorder.

Epilepsy and non-localized brain dysfunction have been invoked, among others, as underlying factors in borderline personality disorder. We have recorded 58 electroencephalograms in 20 borderline patients, first after complete drug washout and then under carbamazepine or placebo double-blind treatment. Taking into account only definite abnormal tracings, we found a 40% incidence of abnormal diffuse slow activity. No patient disclosed focal or epileptiform EEG features. Carbamazepine did not appear to modify the electroencephalogram.

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.

[Alzheimer's disease in 1995]. / La maladie d'Alzheimer en 1995.

Alzheimer's disease is the most common cause of dementia in our society. Recent progress in the understanding of the mechanisms leading to the central nervous system abnormalities produced by this disease will lead soon to new strategies of treatment and new diagnostic approaches.

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.

Effects of routine hyperventilation on PCO2 and PO2 in normal subjects: implications for EEG interpretations.

There are few data in the EEG literature describing the time course of hyperventilation-(HV) induced changes in blood gases, despite this being a routine activating procedure. We studied changes in blood gases and EEG in nine normal adult subjects before, during, and after HV. The mean PCO2 fell 18 mm Hg from the baseline during HV and recovered in 7 min. The mean PO2 rose 7 mmHg during HV and fell to 25 mm Hg below baseline 5 min after HV. The PCO2 recovery period is longer than is usually assumed in clinical EEG. The PO2 fall to a nadir at 5 min after the end of HV suggests that close attention should be paid to this period, as is confirmed by the re-buildup seen in moyamoya disease. Despite uniform changes in blood gases, the EEG median power frequency change showed marked variability; on average, it dropped by 1 Hz during HV and returned to baseline within 2 min of resumption of normal respiration. The EEG root-mean-square power showed a 200% increase during HV and also had returned to normal within 2 min.