[Progressive multifocal leukoencephalopathy: a complication in chronic B-cell lymphatic leukaemia]. / Progressieve multifocale leuko-encefalopathie.

BACKGROUND: Progressive multifocal leukoencephalopathy (PML) is a rare infectious cause of sub-acute neurological symptoms, and occurs predominantly in immunocompromised patients. PML is caused by reactivation of the JC virus. CASE DESCRIPTION: A 79-year-old man with a history of chronic B-cell lymphatic leukaemia (B-CLL) presented at our hospital with a neurological deficit of the left side of his body. He was initially diagnosed with a right-hemisphere stroke. Two months later he returned with progressive paresis and on an MRI of the brain we saw an increase in abnormalities of the white matter. On suspicion of PML we conducted PCR for JC virus on cerebrospinal fluid (CSF), which was negative. Histopathological investigations of a brain biopsy confirmed the diagnosis of PML, four months after he first presented. CONCLUSION: PML is a rare cause of sub-acute neurological symptoms. PML can be difficult to diagnose as a PCR of CSF for JC virus in the early stages of PML can give a false negative result. If PML is suspected, histological investigation of a brain biopsy is necessary.

Screening for aneurysm of the abdominal aorta: prevalence in patients with stroke or TIA.

BACKGROUND AND PURPOSE: Population screening for aneurysms of the abdominal aorta (AAA) is still not implemented in any country, despite proven benefit both in decreased mortality and in cost effectiveness. Detecting a subpopulation with higher prevalence of AAA may alter this situation. METHODS: Between 2002 and 2005, all patients with a stroke or transient ischaemic attack (TIA) admitted to the department of Neurology of a community-based hospital were classified according to the Toast criteria and enrolled in a prospective study to assess the diameter of the abdominal aorta. The diameter was assessed by ultrasonography. A written questionnaire and blood tests were used to assess risk factors. RESULTS: The prevalence of AAA amongst the 499 screened patients in the study was 5.8% [95% confidence interval (CI) 5.6-6.0%]. Of the risk factors or Toast criteria, only male gender and age over 59 years correlated significantly with AAA. In the subgroup of 235 men aged over 59 years, the prevalence of AAA was 11.1% (95% CI 10.4-11.8%). CONCLUSION: The prevalence of AAA in men over 59 years of age presenting with a stroke or TIA is nearly twofold increased (11.1%) compared with all patients. Therefore, screening for AAA in this subgroup of patients seems beneficial. However, further studies are needed to confirm this finding and to explore the clinical benefit and cost effectiveness.

Acute cerebellitis in adults: a case report and review of the literature.

BACKGROUND: Acute cerebellitis is a rare disease with the majority of cases described in children. Little is known about the clinical characteristics and outcome in adults. CASE PRESENTATION: A 37-year-old Caucasian woman presented with headache, nausea, and photophobia, and was diagnosed as having a migraine attack. Two days later, she subsequently returned with aggravated headache, dysarthria and horizontal nystagmus. Magnetic resonance imaging (MRI) showed a swollen cerebellum and hydrocephalus and the patient was diagnosed with acute cerebellitis. Cerebrospinal fluid (CSF) examination showed an elevated leukocyte count and protein. Blood serology showed the presence of immunoglobulin M and immunoglobulin G for both Epstein-Barr virus and cytomegalovirus. The patient was treated with dexamethasone and discharged to a rehabilitation center, where she fully recovered. We searched the literature for adult cases of acute cerebellitis. Including our patient, we identified 35 patients with a median age of 36 years. The etiology was unknown in 34% of cases. The most common clinical presentation consisted of headache, nausea/vomiting and ataxia. Six patients presented with only headache and nausea and subsequently returned with cerebellar signs. In 9 cases, the cerebellitis was complicated by hydrocephalus. Half of the patients ended up with neurological sequelae, while follow-up MRI was abnormal in 71%. CONCLUSION: Acute cerebellitis in adults is a rare disorder which mainly presents with headache, nausea/vomiting and ataxia. To diagnose cerebellitis, imaging of the brain (preferably MRI) is required and CSF examination may be necessary to narrow the differential diagnosis. The treatment depends on the widely diverse etiology, and treatment with steroids is recommended in the case of cerebellar oedema and hydrocephalus. Neurosurgical intervention may be necessary to prevent brain herniation.

[Addendum to the Dutch guideline for minor head/brain injury]. / Addendum richtlijn licht traumatisch hoofd-hersenletsel.

- After introduction of the Dutch guideline for 'Care for patients with minor head/brain injury' (LTH guideline) in 2010, the number of CT scans has increased. Some of these scans were for patients with only trivial trauma and may not have been necessary.- In addition, since this guideline was implemented, there have been changes in the use of anticoagulants and platelet aggregation inhibitors. Non-vitamin-K-dependent oral anticoagulants (NOACs) and platelet aggregation inhibitors, or combinations of these, are prescribed more often.- These two factors have led the Netherlands Society of Neurology to initiate a request for modification of the LTH guideline for adults in two ways: (a) identification of minimal or trivial trauma for which no CT scan is required and (b) inclusion of NOACs and platelet aggregation inhibitors, or combinations of these, in the guideline.

Fatal cerebral oedema associated with primary dengue infection.

Encephalopathy associated with dengue fever is considered to be a rare condition in adults. We describe a patient with a primary dengue infection who, in the absence of overt signs of dengue shock syndrome, died due to progressive cerebral oedema. Autopsy findings demonstrated loss of integrity of cerebral vascular endothelium and involvement of complement activation.

Fatal cerebral embolism in a young patient with an occult left atrial myxoma.

We report a young patient with a fatal cerebral embolism from an occult atrial myxoma. The patient died before echocardiography was performed and at autopsy the definite diagnosis was made. Our patient suffered from migraine of increasing frequency. The physical exercise of sexual intercourse was the precipitating factor of this fatal embolism. The importance of early echocardiography is stressed, especially in view of the recent tendency of early and aggressive stroke treatment.

[Thrombosis of the cerebral veins and sinuses in 62 patients]. / Trombose van de cerebrale venen en sinussen bij 62 patiënten.

OBJECTIVE: Analysis of signs, symptoms, course and treatment of patients with cerebral venous and sinus thrombosis (CVST). DESIGN: Retrospective study. SETTING: Neurological departments of Dutch teaching hospitals. METHOD: Analysis of data of patients admitted to the neurological wards of nine teaching hospitals, diagnosed from 1970 to 1990. All patients with a clinical diagnosis of CVST, confirmed by X-ray angiography, magnetic resonance imaging, surgery or autopsy were included. Patients with cavernous sinus thrombosis were excluded. The clinical condition of patients after 3 months was scored on a 4-point scale. RESULTS: Of 93 patients 62 fulfilled the inclusion criteria. Three months after the diagnosis 41 patients (66%) had recovered, 10 patients (16%) were seriously disabled, 11 patients (18%) had died. Factors associated with a poor outcome were coma (relative risk 3.5) and hemiparesis (relative risk 2.3) at the time of diagnosis. Signs of cerebral haemorrhage or infarction (by computed X-ray tomography) were associated with disability or death. Anticoagulant treatment was not significantly associated with a better or poorer outcome in these patients. CONCLUSION: This study confirms the variations in causes, clinical presentation, natural history, and treatment of CVST. No conclusion could be drawn about the effect of anticoagulant treatment.

Cerebral venous and sinus thrombosis with cerebrospinal fluid circulation block after the first methotrexate administration by lumbar puncture.

We report a patient treated for small lymphocytic lymphoma/leukemia with cerebral venous and sinus thrombosis (CVST) after lumbar puncture with intrathecal administration of methotrexate (MTX). He also developed a cerebrospinal fluid flow block. This is the first report of an association between lumbar puncture and intrathecally administered MTX and the development of CVST. Intrathecal treatment in this patient was discontinued and he was successfully treated with high-dose low-molecular-weight heparin subcutaneously.