Selective bilateral hippocampal lesions after theophylline-induced status epilepticus causes a permanent amnesic syndrome.

Theophylline is known to increase the risk of epileptic seizures and might have a role in seizure-induced brain damage. We present a 55-year-old man who developed an amnesic syndrome after status epilepticus, caused by accidental theophylline intoxication. Imaging studies revealed acute, selective bilateral hippocampal damage, which corresponded to severe disturbances in bilateral temporal functions on neuropsychological testing. Three months later, the memory deficits persisted, while imaging exhibited bilateral atrophy of the hippocampus. Upon his long-term, 18-month follow-up, the patient demonstrated improvements in his daily living abilities, despite the persistence of bilateral temporal deficits. This report provides evidence that theophylline has the potential to provoke permanent seizure-induced neural damage, presumably via inhibition of adenosine receptors, and especially in vulnerable regions of the brain, such as the hippocampus.

[Cognitive impairment in patients suffering from fibromyalgia. An underestimated problem]. / Schmerz und Aufmerksamkeit. Kognitive Defizite bei Fibromyalgiepatienten.

The primary symptom of fibromyalgia (FM) is chronic, widespread pain accompanied by diffuse tenderness to light palpation. However, many patients report a multitude of additional complaints and symptoms. Besides fatigue, exhaustibility and stiffness, a decrease in concentration and memory are further complaints that add significantly to the degree of suffering. This complaint, often termed "fibrofog", is increasingly recognised as an independent symptom that has made its way into the medical literature under the term "dyscognition". Nonetheless there are only a few studies that have specifically investigated neuropsychological deficits in FM patients in order to further specify clinical complaints. The studies performed so far have provided increasing evidence that FM patients have attention and working memory deficits, which are most prominent when patients have to cope with an additional source of distraction. With this review we intend to give an overview of the neuropsychological studies in FM performed so far and to assess possible implications for the underlying pathophysiology. In addition, we discuss potential clinical approaches to these symptoms. A systematic literature review up to June 2009 was carried out using the keywords (pairs) "fibromyalgia" and "cognition", "fibromyalgia" and "dyscognition", "fibromyalgia" and "cognitive deficits".

Neuropsychological profile of adult patients with Niemann-Pick C1 (NPC1) mutations.

Niemann-Pick type C disease is a fatal neurovisceral disorder linked to dysregulation in cholesterol processing. A medication for this disease is currently being tested in clinical trials. However, there is a lack of information on neuropsychological testing parameters for this disease. One aim of this pilot study was to evaluate a test battery that could be used to assess cognitive deficits in different stages of the disease. A second aim was to determine whether specific functional deficits are associated with certain disease stages. Eight men and two women (19-40 years of age) harbouring mutations in the gene coding for the cholesterol trafficking protein NPC1 were put through the same test battery independently of their disease stage. The external staging criterion was based on a five-step clinical scale. Trail Making tests A & B and verbal fluency were sensitive indicators at early stages of NPC. Corsi Block-Tapping, Mini Mental Status, Find Similarities and Clock Drawing showed abnormal results in patients with advanced disease. The Grooved Pegboard, Trail Making and Mosaic tests were unsuitable in advanced disease due to impaired fine motor skills. We observed that visuospatial working memory was less affected by the neurodegenerative process than verbal working memory. The series of tests used here could be supplemented by the severe impairment battery and Raven matrices tests for patients with advanced disease.