Peri-ictal water drinking and other ictal vegetative symptoms: Localizing and lateralizing the epileptogenic zone in temporal lobe epilepsy? Two case reports and review of the literature.

Peri-ictal behavior disorders can be helpful in localizing and lateralizing seizure onset in partial epilepsies, especially those originating in the temporal lobe. In this paper, we present the case of two right-handed women aged 36 and 42 years who presented with partial seizures of mesial temporal type. Both of the patients had drug resistant epilepsy and undergone presurgical evaluation tests including brain magnetic resonance imaging, video-EEG monitoring and neuropsychological testing. The two patients had hippocampal sclerosis in the right temporal lobe and exhibited PIWD behavior concomitant with right temporal lobe discharges documented during video-EEG recordings. Anterior temporal lobectomy was performed in one case with an excellent outcome after surgery. The patient was free of seizures at 3 years follow-up. We reviewed other publications of peri-ictal autonomic symptoms considered to have a lateralizing significance, such as peri-ictal vomiting, urinary urge, ictal pilo-erection. Clinicians should search for these symptoms, even if not spontaneously reported by the patient, because they are often under-estimated, both by the patients themselves and by physicians. Additionally, patients with lateralizing auras during seizures have a significantly better outcome after epilepsy surgery than those without lateralizing features.

[T-cell lymphoma revealed by a mononeuritis multiplex: case report and review of literature]. / Lymphome T révélé par une mononeuropathie multiple: étude d'un cas avec revue de la littérature.

INTRODUCTION: Lymphoma occasionally affects the peripheral nervous system. Neuropathy usually appears in patients with known lymphoma but rarely represents the initial manifestation of underlying malignancy. We report a case in which mononeuritis multiplex (MM) was the dominant feature in the clinical presentation of a peripheral T-cell non-Hodgkin lymphoma (NHL). OBSERVATION: A 32-year-old man suffered from an asymmetric progressive sensory-motor peripheral neuropathy. The left peroneal nerve was affected first, then the left median nerve after one month, followed by the left trigeminal nerve ten months later. The electrophysiological study confirmed the diagnosis of axonal sensory-motor MM. Mediastinal adenopathies, splenomegaly, pancytopenia and inflammatory syndrome were also found. An osteo-medullary biopsy showed a T-cell NHL. Nerve biopsy study found an inflammatory lymphoid infiltration without malignant cell supporting the hypothesis of an inflammatory pathogenic process. Chemotherapy including cyclophosphamide, hydralazine, vincristine and prednisone were administered monthly during 8 months. No improvement was obtained. DISCUSSION: It must be emphasised that this case is an uncommon one. On the one hand, NHL is rarely associated with MM and on the other hand, it can exceptionally be revealed by a MM. We were able to find 30 reported cases of distal neuropathy revealing a NHL including, 8 mononeuritis simplex, 9 MM and 13 polyneuropathies. Polyradiculoneuritis cases were excluded from this study because the neuropathy is usually caused by a meningeal infiltration. The neuropathy was in the majority of the cases chronic and axonal. The lymphoma was more often B-cell than T-cell. The B-cell lymphoma was frequently associated with a poor prognosis. All mechanisms were present with a predominance of neurolymphomatosis.

[Acute Guillain-Barré-like polyradiculoneuritis revealing acute systemic lupus erythematosus: two case studies and review of the literature]. / Polyradiculonévrite aiguë de type Guillain-Barré révélant un lupus érythémateux aigu disséminé: étude de deux cas et revue de la littérature.

The involvement of the peripheral nervous system in systemic lupus erythematosus (SLE) is rare and is dominated by distal symmetric axonal polyneuropathy and multiple mononeuropathy. It usually occurs in late course of the disease. Acute polyradiculoneuropathy of Guillain-Barré syndrome type is very rare and can frequently constitute the first symptom of systemic lupus. We report two cases of acute inflammatory demyelinating polyradiculoneuropathy (AIDP) complicated by respiratory failure due to systemic lupus. In the first case, the pure motor AIDP was the first manifestation of the SLE. The outcome under prednisone treatment was dramatically good with regression of clinical deficit and normalisation of nerve conduction within one month and 12 months of treatment respectively. In the second case the AIDP occurred only one week after diagnosis of SLE and corticotherapy. It was a demyelinating sensory-motor neuropathy. Clinical improvement was obtained after two cures of intravenous gammaglobulin (IVIg). The normalisation of nerve conduction was obtained within 8 months. AIDP is a very rare complication of SLE, but it should be searched as an aetiology of Guillain-Barré syndrome associated to systemic clinical symptoms or to blood inflammation. Corticotherapy could be sufficient, but in some cases the addition of IVIg or plasmapheresis might be necessary.

[Chronic inflammatory demyelinating polyradiculoneuropathy with hypertrophy of spinal roots, brachial plexus and cranial nerves]. / Polyradiculoneuropathie inflammatoire démyélinisante chronique hypertrophique avec atteinte des nerfs crâniens: à propos de deux observations.

We report two patients who presented an atypical chronic inflammatory demyelinating polyradiculoneuropathy with massive nerve root and brachial plexus hypertrophy, and pseudotumoral supraclavicular mass. They also presented an hypertrophy of oculomotor and trigeminal nerves causing an exophthalmos and ocular palsy. Spinal root enlargement and cranial nerve hypertrophy was demonstrated by CT scanner and MRI. Brachial plexus biopsy showed a similar aspect of sural nerve, with an extensive onion bulb formation and perivascular inflammatory cell infiltration. There was an excellent response to steroids in both patients.

[Epileptic seizures and epilepsy in subacute sclerosing panencephalitis (report of 30 cases]. / Crises épileptiques et épilepsie dans le cadre d'une panencéphalite sclérosante subaiguë (à propos de 30 cas).

Subacute Sclerosing Panencephalitis (SSPE) is becoming less frequent in Morocco since the generalization of measles vaccination in 1982. The aim of this study was first to analyze the semiological and elecrophysiological profiles of epilepsy in SSPE in both 'disease-revealing' seizures and sequellar ones, and second, to study the evolution of epilepsy and its possible prognostic value in SSPE. Among the neurological manifestations of SSPE, epilepsy is not as rare as frequently reported in the literature. In this longitudinal series concerning 70 cases of SSPE, 30 developed epilepsy. In two-thirds of our patients the epileptic seizures started in the first year of evolution; they revealed the SSPE in 23% of the cases and were sequellar in the rest. Seizures revealing the SSPE were widely dominated by partial seizures, secondarily generalized or not (86%), suggesting a focalized encephalitic process. Conversely, sequellar seizures were in most cases generalized tonic-clonic (43%), and therefore compatible with an already spread process. The EEG contributed both to the diagnosis of SSPE and to that of the epilepsy, showing epileptic abnormalities in ten patients. The outcome of epileptic seizures was very favorable under antiepileptic drugs, while that of SSPE remained severe and not modified by epilepsy. The authors underline the relative frequency of epilepsy in SSPE, the interest of the distinction between revealing and sequellar seizures, the good prognosis of epilepsy under adequate therapy, and the absence of prognostic value of epilepsy in SSPE.

[Relapsing polyneuropathy in the post-partum period]. / Polyradiculonévrite à rechutes du post-partum.

A patient had five relapses of polyneuropathy: four developed during post-partum. The rapid onset of symptoms with subsequent complete recovery are in favor of a recurrent Guillain-Barré syndrome rather than a chronic relapsing inflammatory polyneuropathy.

Precise interferometric length and phase-change measurement of gauge blocks based on reproducible wringing.

A modern fringe-pattern-analyzing interferometer with a resolution of 1 x 10(-9) and without exclusion of systematic uncertainties owing to optic effects of less than 1 nm was used to test a new method of interferometric length measurement based on a combination of the reproducible wringing and slave-block techniques. Measurements without excessive wringing film error are demonstrated for blocks with nominal lengths of 2-6 mm and with high surface flatness. The uncertainty achieved for these blocks is less than 1 nm. Deformations of steel gauge blocks and reference platens, caused by wringing forces, are investigated, and the necessary conditions for reproducible wringing are outlined. A subnanometer uncertainty level in phase-change-correction measurements has been achieved for gauge blocks as long as 100 mm. Limitations on the accuracy standard method of interferometric length measurements and shortcomings of the present definition of the length of the material artifact are emphasized.

Vitamin E deficiency ataxia associated with adenoma.

Vitamin E is one of the most important lipid-soluble antioxidant nutrient. Severe vitamin E deficiency (VED) can have a profound effect on the central nervous system. VED causes ataxia and peripheral neuropathy that resembles Friedreich's ataxia. We report here a patient presenting this syndrome, but also a prolactin and FSH adenoma. Both the neurological syndromes and the adenoma regressed after treatment with alpha-tocopherol. Although, the presence of the prolactinoma in this patient may not be related to his vitamin E deficiency, alpha-tocopherol treatment seems to be beneficial and might usefully be tested in patients with hypophyseal secreting other forms of adenoma.

Toward subnanometer uncertainty in interferometric length measurements of short gauge blocks.

A primary-level comparator, with a reproducibility of 0.2 nm and intended for realization of a Systeme International length unit in the range of 1-100 mm, is reported. High-precision differential measurements of phase change on reflection from blocks and end plates are demonstrated. A set of experiments has been developed to measure systematic error associated with nonideal interferometer optics and deviations from flatness of an auxiliary plate. For specially selected high-grade 6-mm blocks, reproducible wringing has been achieved with a random uncertainty in length measurements of 0.1-0.2 nm. Subnanometer wear-off of the blocks as a result of the cleaning has been detected. Under the conditions of reproducible wringing, the accuracy of the length measurements is evaluated to be in the 2-3-nm range for 6-mm blocks.

[Dermatomyositis and Wilson disease]. / Dermatomyosite et maladie de Wilson.

A 15 year-old girl developed both a dermatomyositis and a Wilson's disease. A clinical remission was obtained with steroids and D-penicillamine. The potential role of cupric intoxication in the pathogeny of the muscular syndrome is discussed.