D. Saudeau

Sleep and brain lesions: a critical review of the literature and additional new cases

We present a comprehensive review of sleep studies performed in patients with brain lesions complemented by 16 additional personal selected cases and by discussion of the corresponding animal data. The reader is cautioned about the risk of establishing an erroneous correlation between abnormal sleep and a given disorder due to the important inter and intra variability of sleep parameters among individuals. Salient points are stressed: the high frequency of post-stroke sleep breathing disorders is becoming increasingly recognised and may, in the near future, change the way this condition is managed. Meso-diencephalic bilateral infarcts induce a variable degree of damage to both waking and non-REM sleep networks producing and abnormal waking and sometimes a stage 1 hypersomnia reduced by modafinil or bromocriptine, which can be considered as a syndrome of cathecholaminergic deficiency. Central pontine lesions induce REM and non-REM sleep insomnia with bilateral lateral gaze paralysis. Bulbar stroke leads to frequent sleep breathing disorders. Polysomnography can help define the extent of involvement of various degenerative diseases. Fragmented sleep in Parkinsons disease may be preceded by REM sleep behavioural disorders. Multiple system atrophies are characterised by important sleep disorganization. Sleep waking disorganization and a specific ocular REM pattern are often seen in supra-nuclear ophtalmoplegia. In Alzheimer patients, sleep perturbations parallel the mental deterioration and are possibly related to cholinergic deficiency. Fronto-temporal dementia may be associated with an important decrease in REM sleep. Few narcoleptic syndromes are reported to be associated with a tumour of the third ventricle or a multiple sclerosis or to follow a brain trauma; all these cases raise the question whether this is a simple coincidence, a revelation of a latent narcolepsy or, as in non-DR16/DQ5 patients, a genuine symptomatic narcolepsy. Trypanosomiasis and the abnormal prion protein precociously after sleep patterns. Polysomnography is a precious tool for evaluating brain function provided it is realised under optimal conditions in stable patients and interpreted with caution. Several unpublished cases are presented: one case of pseudohypersomnia due to a bilateral thalamic infarct and corrected by modafinil, four probable late-onset autosomal recessive cerebellar ataxias without sleep pattern anomalies, six cases of fronto-temporal dementia with strong reduction in total sleep time and REMS percentage on the first polysomnographic night, one case of periodic hypersomnia associated with a Rathkes cleft cyst and four cases of suspected symptomatic narcolepsy with a DR16-DQ5 haplotype, three of which were post-traumatic without MRI anomalies, and one associated with multiple sclerosis exhibiting pontine hyper signals on MRI.

Recurrence of Spontaneous Subdural Haematoma Revealing Acquired Haemophilia

Introduction Acquired haemophilia is an exceptional haematological disease, with a prevalence of about 1 case per million population per year, affecting predominantly the elderly (people between 60 and 80 years old), and due to the development of auto-antibodies against factor VIII:C (FVIII) [1]. Frequent revealing symptoms are muscle or skin haematomas, subglottic haemorrhages, retroperitoneal bleeding or bleedings in the gastro-intestinal and genitourinary tracts. These haemorrhagic complications can lead to death in 15–20% of cases, despite heavy immunomodulating therapy [1, 2]. Neurological complications are exceptional in this disease, especially spontaneous subdural haematomas (SDH) [1, 2].

A spinal cord intravascular lymphomatosis with exceptionally good outcome

Intravascular lymphomatosis (IVL) is a rare form of high-grade malignant non-Hodgkin’s lymphoma, characterized by the proliferation of neoplastic lymphoid cells within the lumen of small-caliber blood vessels, producing localized vascular occlusion. CNS involvement is found in 75 to 85% of cases.1,2⇓ The clinical presentation is variable and can mimic other neurologic conditions.3 We report a patient with IVL presenting with an isolated, rapidly progressive spinal cord syndrome. A 71-year-old retired woman was admitted because of rapidly progressive paraparesis, accompanied by dysuria and urinary incontinence. At admission, neurologic examination revealed paraparesis with a pyramidal syndrome, T12 hypoesthesia, and anal hypotonia. The rest of the examination was normal. Laboratory studies showed an elevated erythrocyte sedimentation rate and C-reactive protein. Spinal MRI revealed an increase in the size of the conus medullaris and a high signal in T2-weighted sequences. There was no gadolinium enhancement in T1-weighted sequences. Spinal ischemia was diagnosed. Cardiac and aortic ultrasonography were normal and a spinal arteriography …

Création d'une filière neuro-vasculaire régionale : évaluation de la prise en charge à 18 mois

Resume Introduction La prise en charge des patients ayant un accident vasculaire cerebral (AVC) dans les unites neuro-vasculaires (UNV) diminue la proportion de deces et de handicap, independamment de l’acces aux traitements d’urgence comme la thrombolyse intraveineuse. Au CHU de Tours, une filiere d’accueil direct des patients atteints d’AVC permettant l’utilisation du traitement par thrombolyse intraveineuse a ete mise en place en juin 2003. Methodes Nous avons conduit une etude prospective pendant 18 mois, pour evaluer le fonctionnement de la filiere neuro-vasculaire, en recueillant les donnees de prise en charge et de devenir de tous les patients qui y etaient admis. Dans un second temps, ces donnees ont ete comparees a celles recueillies pendant 6 mois aux urgences en 2002, pour les patients suspects d’AVC. Nous avons egalement etudie les conditions d’utilisation du traitement par thrombolyse intraveineuse. Resultats Trois cent soixante-quatre patients ont ete inclus dans l’etude. La creation de la filiere s’est accompagnee d’une diminution significative des delais d’admission des patients et des delais d’imagerie, et d’une plus grande implication des services de transport d’urgence (SAMU). La proportion de patients ayant une mauvaise evolution est moindre dans la filiere, cette diminution ne restant significative apres ajustement que pour les patients ayant un AVC hemorragique. Les conditions d’utilisation du traitement thrombolytique dans notre centre sont comparables a celles de la litterature en terme de securite et d’efficacite. Conclusion Cette etude montre que la creation d’une filiere neuro-vasculaire d’accueil direct et de thrombolyse s’est globalement accompagnee d’une amelioration de la qualite des soins, avant meme la creation d’une unite neuro-vasculaire repondant aux recommandations.

Two Mesencephalic Lacunar Infarcts Presenting as Claude’s Syndrome and Pure Motor Hemiparesis

Two exceptional cases of mesencephalic lacunar infarcts located both in the anterior vascular territory are reported. In patient 1, the infarct selectively involved the red nucleus, thus resulting in a Claudes syndrome. In patient 2, the lesion was limited to the external 2/3 of the cerebral peduncle, and was responsible for a pure motor hemiplegia (PMH). CT scan easily demonstrated the lesion in both cases. Claudes syndrome is very unusual, and PMH has only been reported once before in a mesencephalic infarct. The reasons why these lesions are so uncommon are discussed.

Abrupt onset of disturbed vigilance, bilateral third nerve palsy and masturbating behaviour: a rare presentation of stroke.

The clinical presentation of stroke usually includes sensory–motor impairment, cranial nerve palsies, or cognitive dysfunction. Disorders in behaviour are less frequently seen. The case of a patient with a very disturbing presentation, which included a disturbance in vigilance, bilateral third nerve palsy and masturbating behaviour, is presented. The topography of the lesions and its implications on the deficits observed are discussed.

Exceptional stroke-like episodes in a patient with type I autosomal angioedema

We report on a man with type I hereditary angioedema (HAE) who presented with repeated transient neurologic deficits, totally regressive after a specific C1-inhibitor (C1 INH) injection. Hereditary angioedema, first described in 1888,1 is an autosomal dominant disease, affecting up to 1/50,000 persons, defined in type I as a C1 INH deficiency.2 It is clinically characterized by recurrent edematous crisis at various body sites: mainly relapsing skin swellings, abdominal pain attacks, and life-threatening upper airway obstructions. Neurologic manifestations are exceptionally reported in this affection.3 ### Case report. A 61-year-old man was admitted for the sudden onset of a right sensory-motor deficit. Since childhood, he had had serpiginous erythema and recurrent abdominal pain attacks. The diagnosis of HAE type I was established when he was 14 years old. The disease was still active in 2008 (2 abdominal or respiratory attacks during 3 or 4 days/month), despite a high dose of prophylactic treatment with danazol (600 mg/day). He was an active smoker (40 packs/year) and had a dyslipidemia probably due to danazol. He had no arterial hypertension. At admission, the deficit was …

Internal carotid artery dissection after tonsillectomy in an adult woman.

Glasgow Coma Scale score was E3 V1 M6, the blood pressure 130/70 mm Hg, the pulse 80/min, and she was apyretic. The NIH Stroke Scale score was 25. Throat examination revealed a mild inflammation with swelling of the peritonsillar area. Blood examinations were normal. Brain MRI with diffusion-weighted images demonstrated a large cerebral infarction in the left middle cerebral artery territory, MR angiography showed an irregular stenosis of the left ICA extending from the bulb to the base of the skull. The MR axial scans looking for mural hematoma were noninterpretable due to the clouding of consciousness and agitation, but CT angiography showed widening of the external diameter of the ICA with a crescentic hypodensity around the enhanced narrowed artery ( fig. 1 ) consistent with a mural hematoma. Ultrastructural examination of the skin did not show abnormalities of the elastic tissue, and Ehlers-Danlos syndrome, Marfan syndrome, polycystic renal syndrome, 1-antitrypsin deficiency and fibromuscular dysplasia were excluded. The MR angiography performed 3 months after the onset of symptoms showed recanalization of the left ICA with a slight persistent narrowing of the artery ( fig. 2).

G - 46 Hémosidérose superficielle hémisphérique unilatérale : une présentation atypique

Introduction L’hemosiderose superficielle (HS) est due a un saignement sous arachnoidien repete dont la cause reste inconnue dans la moitie des cas. Classiquement, elle se presente par une ataxie, une hypoacousie et des signes pyramidaux. Observation Un patient, âge de 84 ans, droitier, ancien medecin, fut hospitalise pour une deterioration cognitive evoluant depuis plusieurs semaines, associee a des troubles de la marche, un syndrome dysexecutif essentiellement visuo-constructif, et des hallucinations visuelles elaborees. Les explorations biologiques, et le scanner cerebral, etaient normaux. Il existait une augmentation isolee des hematies (250/mm 3 ) dans le LCR. L’EEG montrait une souffrance hemispherique droite. L’IRM retrouva une hemorragie sous arachnoidienne subaigue hemispherique droite, et des depots spontanement hyperintenses en T1 caracteristiques d’une hemosiderose superficielle droite diffuse. Aucune lesion sous jacente ne fut detectee et le deces survint quelques semaines plus tard. Discussion Les symptomes de l’HS resultent de depots d’hemosiderine dans la region cerebelleuse et medullaire haute. Le scanner cerebral, et le LCR peuvent etre normaux. L’aspect typique de l’IRM en T1 et echo de gradient pose le diagnostic. On peut proposer un traitement par chelateur en fer mais seule l’ablation de la cause est efficace. Les explorations etiologiques doivent etre completes chez le sujet jeune. Conclusion L’HS peut etre hemispherique, unilaterale, et entrainer une encephalopathie severe avec troubles psychiatriques. En l’absence d’etiologie retrouvee, la lesion hemorragique peut rester evolutive et l’issue fatale.

230 Unexpected low prevalence of atrial fibrillation in cryptogenic ischemic stroke: a prospective continuous monitoring study

Introduction Ischemic stroke (IS) is a frequent pathology, burdened by high rate of recurrence and significant morbidity and mortality. There are several causes of IS, affecting prognosis, outcomes and management, but in many cases the etiology remains undetermined despite comprehensive research. We hypothezised that atrial fibrillation (AF) was largely involved in this pathology but was underdiagnosed by standard methods. The aim of this study was to determine the incidence of AF in cryptogenic IS, by using continuous monitoring of the heart rate over several months. The secondary objective was to test the predictive value of atrial vulnerability study towards spontaneous AF. Methods and results We prospectively enrolled 24 patients under 75 years: 15 men and 9 women of mean age 48.8± 13.6 years who experienced cryptogenic IS presumed of cardioembolic mechanism within the last 4 months. Any cause of IS was excluded by normal 12-lead ECG, 24-hour Holter monitoring, echocardiography, cervical Doppler, haematological and inflammatory tests. All patients underwent electrophysiological study. 7 patients (29.2%) had inducible arrhythmia during programmed atrial stimulation and 9 (37.5%) had a latent vulnerability index (LVI) Conclusion This study shows that contrary to what was expected, AF does not appear to be a common pathology in patients under 75 years with unexplained IS. The use of ILR should not be generalized in the systematic assessment of these patients because of its unprofitability. Finally this study attests the poor value of atrial vulnerability study for predicting spontaneous AF in such patients.