Transient global amnesia caused by painless aortic dissection.

Neurological syndromes secondary to acute aortic dissection (AAD) are uncommon and usually consist of focal deficits after an embolic cerebral infarction. This article reports the observation of an AAD with the chief complaint of transient acute memory impairment-that is, a non-usual stroke-like symptom.

Emergent EEG in clinical practice.

OBJECTIVE: Emergency situations require a rapid and precise diagnostic approach. However, the exact role and value of the electroencephalogram (EEG) in emergent conditions have yet to be clearly defined. Our objective was to determine why clinicians order an emergency EEG, to assess to what extent it helps establish a correct diagnosis and to evaluate the result it has on subsequent patient management. METHODS: We studied all successive emergency EEGs ordered during a 3-month period in our institution. We analyzed the reasons why each EEG was ordered and interviewed the prescribing clinicians in order to determine the impact the result of the EEG had on the diagnosis and subsequent therapeutic management. RESULTS: We prospectively studied a total of 111 consecutive recordings. The main reasons for ordering an emergent EEG were: suspected cerebral death (21%), non-convulsive status epilepticus (19.7%), subtle status epilepticus (14%) and follow-up of convulsive status epilepticus (11.2%). In 77.5% of the cases the clinicians considered that the EEG contributed to making the diagnosis and that it helped confirm a clinically-suspected diagnosis in 36% of the cases. When subtle status epilepticus (SSE) or non-convulsive status epilepticus (NCSE) was suspected, the diagnosis was confirmed in 45% and 43.3% of the cases, respectively. In 22.2% of the requests involving follow-up of convulsive status epilepticus after initial treatment, the EEG demonstrated persistent status epilepticus. It resulted in a change in patient treatment in 37.8% of all the cases. When the EEG helped establish the diagnosis, patient treatment was subsequently modified in 46.6% of the cases. CONCLUSIONS: This prospective study confirms the value of an emergent EEG in certain specific clinical contexts: the management of convulsive status epilepticus following initial treatment or to rule out subtle status epilepticus. An emergent EEG can also be ordered if one suspects the existence of non-convulsive status epilepticus when a patient presents with mental confusion or altered wakefulness after first looking for the specific signs suggesting this diagnostic hypothesis. SIGNIFICANCE: After 50 years of development and use in daily practice, the EEG remains a dependable, inexpensive and useful diagnostic tool in a number of clearly-defined emergency situations.

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.

Epilepsy and language development: the continuous spike-waves during slow sleep syndrome.

BACKGROUND: Continuous spike-waves during slow sleep syndrome (CSWSS) is a rare epileptic syndrome occurring in children, which is characterized by the association of epilepsy, neuropsychological disorders, and abnormal paroxysmal electroencephalographic (EEG) discharges activated by sleep. Language can be affected but, to date, language disorders and their long-term outcome have been documented only rarely. PURPOSES: Description of language impairment in patients with the CSWSS. METHODS: We performed a detailed language testing in 10 right-handed children and adolescents with the CSWSS. Their pragmatic performance was compared to that of a control population of 36 children aged 6-10 years. RESULTS: Patients with CSWSS had lower scores in tests measuring their lexical, morphosyntactic, and pragmatic skills compared to controls. Comprehension remains unaffected. In addition, language impairment was found to be just as severe in patients in remission as those still in an active phase. CONCLUSIONS: We found severe language impairments in lexical and syntactic skills. The language profile is different from that observed in the Landau-Kleffner syndrome. Moreover patients in remission and those in an active phase of the CSWSS have the same language impairment profiles. This confirms the poor long-term neuropsychological prognosis. Our results raise points about the relationship between epileptic activity and language development. This pilot study underscores the need to assess language, and especially pragmatic skills, and to study long-term outcome in such childhood epileptic syndromes.

Atypical language impairment in two siblings: relationship with electrical status epilepticus during slow wave sleep.

We report the case of a young girl who presented severe learning disabilities in oral and written language related to a continuous spike-waves during slow sleep (CSWS) syndrome. A sleep EEG recording obtained in her younger brother, who presented a clinical pattern suggesting developmental dysphasia, also showed a CSWS syndrome. These two clinical cases underscore the need to look for this syndrome in the siblings of an affected child when learning difficulties appear in a child who previously had normal psychomotor development.

Night terrors associated with thalamic lesion.

OBJECTIVE: To describe a case with night terrors (NT) symptomatic of a thalamic lesion. METHODS: Videopolysomnography and brain MRI were used to study a 48 year old woman with a recent onset of brief episodes, occurring exclusively during nocturnal sleep, where she suddenly sat up in bed, screamed and appeared to be very frightened. RESULTS: Videopolysomnography recorded an episode suggestive of NT. Sleep fragmentation with frequent brief arousals or microarousals was also evident mainly during slow wave sleep. The brain MRI showed increased T2 signal from the right thalamus suggestive of a low-grade tumor. CONCLUSIONS: Our case suggests that NT starting in adulthood can, rarely, be symptomatic of neurological disease, and warrant further investigation with MRI. SIGNIFICANCE: A thalamic dysfunction, disrupting at this level the arousal system, may play a role in provoking NT.

Outcome at adulthood of the continuous spike-waves during slow sleep and Landau-Kleffner syndromes.

PURPOSE: The aim of this study was to determine the clinical, social, and/or professional and cognitive outcomes in adulthood of the continuous spike-waves during slow sleep (CSWS) and Landau-Kleffner syndromes, which are two rare epileptic syndromes occurring in children. METHODS: We enrolled seven young adults, five who had a CSWS syndrome, and two, a Landau-Kleffner syndrome in childhood. We evaluated their intellectual level as well as their oral and written language and executive functions. RESULTS: This study confirmed that the epilepsy associated with these syndromes has a good prognosis. Only one patient still had active epilepsy. However, the neuropsychological disorders particular to each syndrome persisted. Only two patients had followed a normal pathway in school. Three of the five patients with a CSWS syndrome during childhood remained globally and nonselectively mentally deficient. We found no evidence of the persistence of a dysexecutive syndrome in this study group. The intellectual functions of the two patients with Landau-Kleffner syndrome were normal; however, their everyday lives were disrupted by severe, disabling language disturbances. We discuss the role of some prognostic factors such as the location of the interictal electric focus and the age at onset of CSWS. CONCLUSIONS: These two epileptic syndromes of childhood are very similar in many respects, but their clinical outcomes in adulthood are different.

Abnormal SMN1 gene copy number is a susceptibility factor for amyotrophic lateral sclerosis.

The etiology of amyotrophic lateral sclerosis remains unknown in the majority of cases. Homozygous SMN1 (survival motor neuron) gene deletion causes spinal muscular atrophy, and SMN2 gene deletions are possible risk factors in lower motor neuron disease. We studied SMN1 and SMN2 genes copy numbers in 167 amyotrophic lateral sclerosis patients and in 167 matched controls. We noted that 16% of amyotrophic lateral sclerosis patients had an abnormal copy number of the SMN1 gene (1 or 3 copies), compared with 4% of controls. An abnormal SMN1 gene locus may be a susceptibility factor for amyotrophic lateral sclerosis.

Ictal bradycardia followed by cardiac asystole: a case report.

We report on a patient with a 30-year history of left temporal lobe epilepsy who presented with ictal bradycardia followed by cardiac asystole. The EEG during the ictal period was documented and analyzed. Clinical features and therapeutic considerations are discussed.