Stroke-like presentation of cerebral lymphoma

INTRODUCTION: The clinical presentation of primary cerebral lymphoma can take on many forms. CASE REPORT: We report the case of a patient who experienced recurrent neurological events mimicking stroke with normal brain MRI. A late performed MRI showed a mesencephalic lesion. A biopsy was obtained and led to the diagnosis of primary B cell lymphoma. CONCLUSION: This observation illustrates the diagnostic challenge of this rare disorder with a poor prognosis.     

Persistent neurological sequelae due to cerebral malaria in a cohort of children from Mali

INTRODUCTION: Several neurological complications are associated with cerebral malaria (CM). However, few long-term data from childhood survivors have been published. METHODS: A cross-sectional study was carried out in Mali among children followed from 1999 to 2002 after serious and complicated malaria. Our aim was to evaluate the persistent neurological sequelae associated with CM. RESULTS: This study concerned 101 subjects who had had CM. Mean age was 5.6+/-3.6 years. Twenty-eight children presented persistent neurological sequelae (27.7p.cent). Among them eight (7.9p.cent) children had developed these sequelae just after CM and 20 (19.8p.cent) a few months later: headaches, mental retardation, speech delay, bucco-facial dyspraxia, diplegia and frontal syndrome (one case each), dystonia (two cases), epilepsy (five cases) and behavior and attention disorders (15 cases). CONCLUSIONS: In this study, we show that neurological signs due to CM can persist in the long run. Long-term follow-up and proper management after CM are essential.     

Primary central nervous system lymphoma presenting with central neurogenic hyperventilation. A case report and review of the literature

INTRODUCTION: Central neurogenic hyperventilation (CNH) in an awake patient is a rare entity. OBSERVATION: We report here a 54-year-old patient who developed central neurogenic hyperventilation as the initial presentation of a primary central nervous system lymphoma located in the brainstem. CONCLUSION: The patient's hyperventilation resolved completely with chemotherapy for primary CNS lymphoma. Most of the cases reported in the literature are related to a diffuse tumor of the brainstem with an intriguing overrepresentation of primary CNS lymphoma. The pathogenesis of CNH is discussed.     

Cerebral angiitis caused by periarteritis nodosa

INTRODUCTION: We report a case of periarteritis nodosa revealed by a cerebral angiitis which recovered under treatment. OBSERVATION: A 52-year-old patient suddenly presented with a left sensory syndrome and a fluctuating aphasia due to ischemia involving both parietal lobes. The diagnosis of periarteritis nodosa was based on the following criteria: severe loss of weight, renal insufficiency, hypertension, angiography suggesting an arteritis. Instead of an ileo-cecal perforation, the patient recovered under corticosteroid and immunosuppressive therapy. CONCLUSIONS: Stroke in periarteritis nodosa may occur early be and associated with a good outcome.     

Cerebral amyloid angiopathy

Cerebral amyloid angiopathy (CAA) is characterized by deposition of cerebrovascular amyloid protein in the media of leptomeningeal vessels. (amyloid B protein, cystatin C, transthyretin, gelsolin, and prion protein). It is a cause of cerebrovascular disorders including cerebral hemorrhage, cognitive impairment and unusually transient neurological symptoms. It is the main contributing factor to cerebral hemorrhage after hypertension in the elderly. We aimed to review epidemiological, pathophysiological and clinical and MRI imaging data in CAA.     

Cerebral hydatic cyst and psychiatric disorders. Two cases

The hydatidosis is an endemic illness in regions of the Middle Orient, Mediterranean, south of America, north Africa and the Australia. The preferential localization of cyst hydatic is the liver (48%), the lung (36%) and in 6% of cases it localizes in unaccustomed place as the brain. Intracerebral localization is relatively rare, its impact is 1 to 5% of all cases of hydatidose. This localization is the child's appendage with a masculine predominance. The cyst hydatic intracranien is often lone, of localization usually supratentorielle, sometimes infratentorielle. Symptoms are especially the diffuse headache associated to various neurological signs in relation with sits of the tumor. The psychiatrics symptoms depends on its localization, sides, intracranial hypertension, and the previous personality. In 15 to 20% of cases these tumors can appear in the beginning of their evolution by the isolated psychiatric symptoms. We report the case of two patients that have been hospitalized first in the Academic Psychiatric Unit of Marrakech for isolates psychiatric disorders and whose scanning revealed the presence of cerebral hydatic cyst and that required a surgical intervention in neurosurgery. Case 1 - Patient 29 years old, bachelor, uneducated, leaving in country outside, fermar, in permanent contact with dogs. No particular medical history. The patient has been brought by his family to the psychiatric emergencies after behavior disorders. The beginning of his symptomatology was one year ago by behavior disorders: instability, violence, isolation, and a corporo-sartorial carelessness. His symptomatology worsened and the patient became very aggressive. In psychiatric unit, he was disregarded, sad, anguished, indifferent to his state, very dissonant, completely detached, depersonalized. He brought back some visual and auditory hallucinations with attitude of monitoring. He was raving with delirium of persecution, of ideas of reference and delirium of bewithment. He was unconscious of his disorders. The patient has first been put under classical neuroleptic 9 mg/day of Haloperidol and 200 mg/day of chlorpromazine. The diagnosis of schizophrenia has been kept according to criteria of DSM IV. The PANSS (Positive and Negative Syndrome Scale) was to 137 (score on a positive scale was to 34, score on a negative scale was to 35 and the general psychopathologie scale was to 58). One week after his hospitalization, he developed headache with subconfusion, a cerebral scanning has been made in emergency and showed a voluminous cyst in oval foramen compressing the mesencephalon strongly. The cyst was well limited, hypodense, not taking the contrast, and without intracerebral oedema, the diagnosis of cerebral hydatic cyst has been made. The complementary exploration didn't show any other localizations, and biologic exam results didn't show any particular anomalies. The patient has been operated in neurosurgery. The immediate evolution was favorable with disappearance of confusion and absence of complications. The patient was lost of view. Six months after, the patient has been readmitted to the psychiatric emergency. He dropped his neuroleptic treatment. He was aggressive, raving, hallucinated and depersonalized. The global score to the PANSS was 63. He has been put back under neuroleptics. Three weeks after improvement and passage of the PANSS to 30, the patient went out. We couldn't have a cerebral scanner of control because the patient had no medical assurance and no money for cerebral scanner. Case 2 - Patient aged of 53 years, father of four children, uneducated, native and resident of Marrakech, confectioner as profession. He is in contact with dogs since 12 years. He has been brought to the psychiatric emergencies by his family after an agitation. The history of his illness seemed to go back at eight months ago, by the progressive apparition of an instability, sleep disorders, hostility, associated with an emotional lability. To the interview he was agitated and had a delirium of persecution. He was convinced that his wife and his children plotted against him. He had sad mood. He was anguished and had auditory and visual hallucinations. The patient was not confused but it had a hypoproxie, an fixing amnesia, a disorders of judgment and a light left hemiparesia. Cerebral scanner revealed three cerebral cyst. The first measuring 42 x 40 mm, sitting at the level parietal right, to the contact of the occipital horn, dragging his/her/its amputation and an effect of mass on ventricle homolateral, the median line and ventricle controlateral. The two other, at the level of the center semi oval, behind the first, measuring 23 mm and 15 mm on the big axis. The patient has been addressed in neurosurgery. He had a completeray exploration to search other localizations. The thoracic x-ray showed 2 pulmonary cyts. The abdominal scan and imagery by magnetic resonance showed liver cyst, peri-heart cyst and mediastinal cyst. The patient has been operated for these three cysts with good recuperation on the psychiatric and neurological symptoms. He has been addressed in heart surgery for the heart localization. The hydatidose is an endemic illness in Morocco and constitute a public health problem. The cerebral localization is rare and appear by signs of cerebral hypertension and signs of focusing. The psychiatric demonstrations are rare but preserve a major interest, by the therapeutic measure specificity that they impose. Of course, the surgical ablation of the tumor can be sufficient to attenuate the psychiatric symptoms but the recourse to a specific treatment can prove to be necessary to act on the precise targets. We are conscious of the methodological difficulties that present these 2 cases but there are unfortunately due to the financial difficulties of our patients.     

Fatigue in neurological disease: different patterns in stroke and multiple sclerosis

INTRODUCTION: Fatigue is a complex, subjective experience, frequent in multiple sclerosis (MS) and stroke patients. The tiredness these patients experience can take on many features depending not only on the cerebral location of the lesions and mood aspects, but also on the pathophysiology of the disease. Thus, it is reasonable to expect that fatigue may have different implications in MS and stroke. The aim of the present work was to compare fatigue syndrome in these two populations. Patients were matched for handicap. MATERIALS AND METHODS: Seventy-nine stroke and 39 MS outpatients were included with the following inclusion criteria: i) patients with possible or relapsing-remitting MS with an Expanded Disability Status Scale (EDSS) score<2.5, disease duration<6 years, and stable medical condition for at least 6 weeks; ii) stroke patients with mild neurological impairment, i.e. scoring<3 at the National Institute of Health Stroke Scale (NIHSS) one year after stroke; iii) absence of functional impairment (Barthel index=100) and similar negligible handicap (Rankin scale<2 for both groups); no or mild cognitive deficit; iv) neither DSMIV criteria of depression, nor significant anxious/depressive symptoms (Hospital Anxiety and Depression scale; HAD; score<8) in both groups. The Fatigue Assessing Instrument (FAI) was used to assess fatigue. RESULTS: Twenty-nine percent of stroke and 46 p. cent of MS patients had a significant score on the FAI (p<0.05). Multiple regression analysis using groups, gender and age as factors showed a group effect in 3 out of 4 subscales: MS patients scored higher than stroke patients mainly for psychic impact (4.86 vs. 3.28), but also for severity (mean 3.86 vs. 2.97) and specificity (4.36 vs. 3.32). Response to rest (5.36 vs. 6.06) only tended to be better in the stroke group. In the subpopulation with significant fatigue scores, psychic impact was more elevated in the MS group. The functional consequence of fatigue in physical, professional and social activities were similar. DISCUSSION: Fatigue was more severe in MS than stroke patients, independently of disability. The most significant factor in the MS group was the psychic impact, reflecting impaired motivation, concentration and irritability, despite the absence of depression. However, subjective consequences of fatigue on work, family and leisure activities were comparable in both groups.     

Listeria monocytogenes encephalitis revealed by left progressive hemiparesis

INTRODUCTION: Central nervous system listeriosis is a diagnostic and therapeutic challenge for the clinician. CASE REPORT: We report the case of a 66-year-old woman who was admitted for a left progressive hemiparesis associated with headaches. She was treated for one year by immunosuppressive drugs for vulgaris pemphigus. At the time of admission, examination revealed left hemiparesis without fever, and a computed tomography brain scan demonstrated a focal lesion in the right frontal lobe. Blood analyses were normal. Two days after, she suddenly developed fever (40 degrees C), and aggravation of her motor deficit followed by partial motor seizures. The cerebrospinal fluid was normal. Treatment with amoxicillin (3g IV q6h), cefotaxim, gentamycin (120mg IV q12h) and aciclovir was started empirically. The brain MRI without gadolinium displayed infiltrative lesions in the right hemisphere and in the mildbrain. The blood culture grew Listeria monocytogenes. The antimicrobial regimen was changed to amoxicillin for seven weeks and gentamicin for the first ten days. Four days after beginning the antimicrobial regimen, the brain MRI with gadolinium displayed several abscesses measuring less than one cm diameter. The clinical and imaging outcome excellent. CONCLUSION: Meningitis is by far the most central nervous system listeriosis. In our patient, the diagnosis of listeria monocytogenes encephalitis was established on the basis of positive blood cultures, as such patients do not have sterile cerebrospinal fluid.     

Cerebral infarction, a complication of paroxysmal nocturnal haemoglobinuria

Paroxysmal nocturnal hemoglobinuria (Marchiafava-Micheli disease) is a rare acquired clonal disorder of the hematopoietic stem cell. Its most frequent clinical manifestations are hemolytic crisis and the most serious are venous thrombosis of the mesenteric, hepatic, portal or cerebral territories. Arterial cerebral infarctions are extremely rare. We report and comment on a case of multiple strokes occurring during an hemolytic crisis and despite effective long-term anticoagulation treatment.     

Neurological aspects of Fabry's disease

Fabry disease is a rare X-linked disorder caused by deficient activity of the lysosomal enzyme alpha-galactosidase A. Progressive accumulation in lysosomes of the undegraded glycosphingolipids leads to a multi-system disease with dermatological, ocular, renal, cardiac, and neurological manifestations. Peripheral nerve involvement, neuropathic pain and chronic acroparesthesiae, are frequent and early-onset signs revealing the disease. They are due to the involvement of small nerve fiber, thus explaining the normality of electroneuromyography. Cochleo-vestibular and autonomic nervous system involvement is frequent. Besides rare aseptic meningitis, central nervous system involvement is essentially represented by cerebrovascular events (stroke, transient ischemic attack). Affecting essentially the posterior circulation, their etiologies have to be clarified: progressive stenosis of small vessels with globotriasocylceramide deposits, arterial remodeling, endothelial dysfunction, pro-thrombotic state, cerebral hypoperfusion consecutive to dysautonaumy, cardiac embolism. MRI shows numerous silent lesions, increasing with age, mainly in small perforant arteries (periventricular white matter, brainstem, cerebellum, basal ganglia). Pulvinar calcifications, due to an increase in cerebral hyperperfusion, could be specific of Fabry disease. Positon tomography analysis shows a reduced cerebral flow velocity and impaired cerebral autoregulation, secondary to the glycosphingolipid storage in vascular endothelial cells. Enzyme replacement therapy has to be carefully monitored.     

The association of cardiac involvement and ischemic stroke in Churg Strauss syndrome

In Churg and Strauss syndrome (CSS), three patterns of neurological involvement can be found, including mono or polyneuropathy, encephalopathy and stroke. We report two cases of stroke associated with major hypereosinophilia and cardiac involvement, leading to a diagnosis of CSS. Neurological and general outcome were good under treatment with steroids in combination with cyclophosphamide in one case. Churg and Strauss syndrome must be considered when a stroke is associated with a cardiac involvement and hypereosinophilia.     

Treatment with intravenous rt-PA in a general hospital context: A review of the Saint-Jean Hospital experience from Perpignan, France

INTRODUCTION: Intravenous recombinant tissue plasminogen activator (rt-PA) has approval for use despite of its authorization for treatment of ischemic stroke within the 3-hour time window in 2003, is rarely used in community hospital (CH). It therefore remains questionable if the positive results of the key studies conducted in specialized centers may be extended to community hospitals less specialized in the management of stroke. METHODS: We report the results of an observational cohort study including 39 patients treated with intravenous rt-Pa (according to the NINDS rt-PA stroke trail treatment protocol) at St Jean Hospital (Perpignan, France) between March 1, 2002 and August 31, 2005. Results are compared to those of the treated arm of the NINDS study. RESULTS: 1.2p.cent of ischemic stroke were treated with intravenous rt-Pa. Results are similar to those of the NINDS study: The outcome was favorable (modified Rankin score (mRS) with 0 or 1) for 44p.cent of the patients (as compared to 39p.cent in the NINDS study (X2 = 0.34; p = 0.5)) and there was no significant difference in term of death or outcome as assessed by mRS at 3 months (X2 = 0.09; p = 0.75 and X2 = 0.77; p = 0.75, respectively). No symptomatic hemmorrhagic transformation related to the use of rt-Pa was observed. CONCLUSION: Our results indicate that rt-PA therapy for ischemic stroke may be as safe and effective in the setting of a community hospital as it is in specialized centers.     

Aseptic cerebral venous thrombosis and multiple cerebral tuberculomas associated with pulmonary miliary tuberculosis

INTRODUCTION: Severe pulmonary tuberculosis may be complicated by deep vein thrombosis due to a state of hypercoagulability. OBSERVATION: We report a case of pulmonary miliary tuberculosis associated with cerebral venous thrombosis and multiple intracranial tuberculomas. A 65-year-old woman developed a confusional syndrome one week after starting treatment for pulmonary military tuberculosis. Neuroimaging reveals a thrombus in the right lateral sinus and multiple silent intracranial tuberculoma. CONCLUSION: The patient was given anticoagulants and fully recovered.     

Results of a survey of different types of stroke care in France: from stroke unit to conventional care

INTRODUCTION: Stroke is a worldwide problem with high incidence, mortality and disability rates. A Meta analysis by the Stroke Unit Trialist Collaboration showed that stroke units improve outcome for stroke patients. Stroke care should take place in stroke units which consist of a hospital unit or part of a hospital unit that exclusively takes care of stroke patients. European recommendations have been published by the European Stroke Initiative and French recommendations by the Societe Fran?aise Neurovasculaire. METHODS: We sent a questionnaire to French stroke physicians in order to establish the different types of stroke care for French inpatients. RESULTS: About half the people surveyed answered (106/193) and we selected 74 responses in order to have only one response for each hospital. Thus, we analysed 74 responses from 74 different French hospitals, coming from the whole country. Of the 74 hospitals, 23 had a stroke unit, 21 had dedicated beds for stroke inpatients, and 30 had no specific care. We identified 5 different types of stroke units: those with intensive acute care and post acute care (8/23), those with intensive acute care that were coordinated with a neurology department (7/23), those with beds in the neurology department (3/23), one with three levels (emergency, acute care and post acute care) and one that shared intensive care with cardiology. Structures of 3 stroke unit couldn't be identified. We studied their conformity to the recommended guidelines and found that, in most cases, they followed them closely. We studied differences between hospitals with stroke units and hospitals without stroke units in terms of technical or human means. We founded only a few differences between these two groups in the advantage of stroke units over hospitals without stroke units. DISCUSSION: We found major differences of organisation between different types of stroke units, but no differences between hospitals with stroke units and other hospitals. These stroke units are generally in conformity with the recommended guidelines.     

Cerebral infarction related to intracranial radiation arteritis twenty-four years after encephalic radiation therapy

We report a case of a resolutive late cerebral ischemic event, related to radiation induced vasculopathy of the left posterior cerebral artery, documented by MRI, situated in the irradiated volume 24 years before, for an astrocytome with malignant potential.     

Primary Gougerot-Sjogren's syndrome presenting as a cerebellar syndrome

Primary Sjogren's syndrome is an autoimmune exocrinopathy with various extraglandular complications. Central neurological manifestations were described in 20 percent of cases. Cerebellar syndrome is uncommon in Sjogren's syndrome, but can occur after diagnosis, or rarely as an inaugural sign of the disease. We report the case of a 54-year-old man who presented a cerebellar syndrome with xerostomy and xerophthalmos. The diagnosis of primary Sjorgren's syndrome was confirmed on the accessory salivary gland biopsy. The patient received oral and intravenous corticosteroid therapy associated with cyclophosphamide monthly. Outcome was good.     

Socio-economic aspects of stroke management

BACKGROUND: Stroke is the third leading cause of death and the first leading cause of disability in developed and developing countries. It is one of the most demanding public health problems to be faced in the upcoming years, particularly because of population aging. STATE OF THE ART: New therapeutic advances in the management of acute stroke have changed our perception of this condition and have had a major impact on healthcare organization and subsequently healthcare expenditures. Care required for the stroke victim is costly in both developing countries and in developed countries. Hemmorhagic events are the most costly, but their prevalence in Western countries is lower than ischemic events. Prevalence of ischemic events is higher in Asian countries. The direct costs of stroke, both for primary and secondary events, constitute the larger part of healthcare expenditures. The mean cost of stroke in France is estimated at 18,000 euros for the first 12 months. Disability accounts for 42 percent of the variable cost of stroke. During the first year, the acute phase accounts for 40 percent of the cost, rehabilitation and mid-term hospitalization for 29 percent, and ambulatory care for 8 percent. After 46 months, the cost of ambulatory care exceeds the cost of the first six months of care during and following the acute phase. CONCLUSION: Any improvement in the primary or secondary prevention of stroke will lead to a decrease in the incidence and prevalence of stroke, and any therapeutic advance capable of reducing disability will consequently reduce the overall cost of stroke.     

Cerebral venous thrombosis secondary to essential thrombocythemia

INTRODUCTION: Essential thrombocythemia (ET) is a myeloproliferative syndrome; cerebral venous thrombosis (CVT) is a rare complication. OBSERVATION: We report the case of a 20-year-old woman with an uneventful history who was admitted with intracranial hypertension syndrome which had developed over the last four months in association with bilateral decline of visual acuity. Physical examination at admission revealed stage II papilloedema and absence of any focal neurological signs. The brain MRI and the venous MRA showed a thrombosis involving the superior longitudinal sinus and the lateral sinus. The etiological analysis disclosed essential thrombocythemia (ET). The patient was given an antiedema and anticoagulant treatment. Later, an etiological cytoreductive therapy was initiated. Signs of intracranial hypertension regressed progressively with persistence of acute visual disorders associated with sequelar optical atrophy. Discussion. ET is a rare cause of CVT. Reports in the literature have discussed the mechanisms, the physiology, the therapeutic modalities and the clinical course of these CVTs secondarily to ET. CONCLUSION: Stroke, especially transient ischemic attack, is the usual thrombotic expression of myeloproliferative syndrome. CVT is much more exceptional.     

Management of malignant gliomas diagnosed during pregnancy

INTRODUCTION: Glioma is seldom diagnosed during pregnancy. In this situation management presents difficult problems for both neuro-oncologists and obstetricians. We report four cases and discuss the management of this unusual situation. CASE REPORT: The first patient was admitted to hospital at 29 weeks' gestation because of a generalized seizure and a right hemiparesis. MRI showed a left fronto-insular lesion. A stereotactic biopsy was obtained and revealed an anaplastic oligodendroglioma. With corticosteroids the patient remained stable until cesarean delivery at 36 weeks. In post-partum additional treatment with chemotherapy was started. The second patient was hospitalized at 26 weeks' gestation because of cranial hypertension, right hemiparesis and aphasia. MRI showed an important left fronto-parietal lesion. Partial resection was performed at 28 weeks. Histology revealed a glioblastoma multiforme. With corticosteroids the patient remained stable until cesarean delivery at 33 weeks. In post-partum additional treatment with radiotherapy and chemotherapy was started. The third patient was admitted to the hospital at 12 weeks' gestation because of cranial hypertension. MRI showed a left frontal lesion. A subtotal resection was done at 13 weeks. Histology revealed a glioblastoma multiforme. Two weeks after surgery the patient's neurological condition worsened and in agreement with the patient a therapeutic abortion was decided. Afterwards additional treatment with radiotherapy and chemotherapy was started. The last patient received combined treatment with radiotherapy and chemotherapy for local recurrence of a mesencephalic high-grade glioma. A posteriori it was discovered that the patient was at 4 months' gestation during this treatment. Cesarean delivery was done at 36 weeks. The child was normal at birth and is still in good health 5 years later. CONCLUSION: The management of gliomas diagnosed during pregnancy should not be different from the standard management of gliomas in young non-pregnant adults. Pregnant women because of their young age can have a long survival. Their pregnancy should not prevent them from receiving the best treatment for their glioma. Treatment will depend upon clinico-radiological presentation, histology, gestational age and the patient's desires. Generally speaking, surgical resection of high-grade gliomas should not be delayed during pregnancy. Progress in anesthesia and neurosurgery have greatly reduced the risks for the foetus. After delivery, if the delay between surgery and delivery is too long it is possible to begin cerebral radiotherapy during pregnancy. After the first trimester of gestation this treatment can be given without any important risks for the child.     

Wolfram's syndrome presenting as a cerebellar ataxia

INTRODUCTION: Wolfram syndrome is a genetic disease with recessive autosomic transmission, associating early-onset diabetes mellitus and bilateral optical atrophy. CASE REPORT: We report the case of a 47-year-old patient for whom we diagnosed a Wolfram syndrome in view of a late neurological syndrome in association with ataxia and bilateral horizontal nystagmus. The brain resonance magnetic imaging revealed a major atrophy of the brainstem and cerebellum. CONCLUSION: Wolfram syndrome is a rare pathology, with fatal consequences before the age of 50. The association of diabetes mellitus and optical atrophy, especially when there are other symptoms (ataxia, deafness, diabetes insipidus, neuropsychiatric manifestations or urinary tract disorders) should lead to this diagnosis and to carry out a genetic confirmation.