Ischemic stroke of unusual cause: clinical features, etiology and outcome

The clinical features, etiology and neurological outcome of ischemic stroke of unusual cause (ISUC) have rarely been reported. We retrospective reviewed all patients with this stroke subtype entered in the Sagrat Cor Hospital of Barcelona Stroke Registry, which includes data from 2000 consecutive first-ever stroke patients admitted to the hospital between 1986 and 1995. Patients with previous ischemia and/or hemorrhagic stroke were excluded. Topographic, anamnestic, clinical and neuroimaging characteristics of ISUC were assessed. Predictors of this stroke subtype were determined by logistic regression analysis. Ischemic stroke of unusual etiology was diagnosed in 70 patients (32 men and 38 women), with a mean +/- SD age of 52 +/- 22.4 years. This stroke subtype accounted for 4.3% of all first-ever strokes and 6% of all first-ever brain infarcts. Etiologies included hematological disorders in 17 cases, infection in 11, migraine stroke in 10, cerebral infarction secondary to venous thrombosis in nine, primary inflammatory vascular conditions in six and miscellaneous causes in 17. In the multivariate analysis after excluding cerebral venous thrombosis (n = 9) and arterial dissection (n = 4), because of typical clinical and radiological features, independent predictors of ISUC included 45 years of age or less (odds ratio [OR] 14.8), seizures (OR 6.8), headache (OR 5.2), hemianopia (OR 2.6) and occipital lobe involvement (OR 3.0). Patients with ISUC presented a lower in-hospital mortality rate (7.1% vs. 14.4%; P < 0.05), were more frequently symptom free at discharge (35.7% vs. 25.80%; P < 0.05) and experienced a longer mean length of hospital stay (23.7 days vs. 18.2 days; P = 0.06) than non-ISUC patients. We conclude that ISUC is infrequent, etiologies are numerous and hematologic disorders are the most frequent cause. We emphasize the better prognosis and the need to distinguish it from other ischemic stroke subtypes which have a different treatment approach and outcome.     

Cerebrovascular diseases in patients aged 15 to 40 years: neuropathological findings in 47 cases]

Cerebrovascular diseases in patients between 15 and 40 years old are not a frequent subject in Latin-American literature, especially when focusing on neuropathology. We analyzed 47 brains from necropsies performed from 1987 to 1997 and selected on a basis of age and the presence of vascular pathology. From the 47 analyzed brains, 26 belonged to females (55.3%). When distributed among age groups, 12.8% (n=6) affected patients from 15 to 20 yo, 51,1% (n=24) from 21 to 30 yo, 36,2% (n=17) from 31 to 40 yo. The underlying diseases were: cardiac and haemathologic (19.2%), pregnancy complications (12.76%), infections, diseases of blood vessels and neurological (10.1% each) amongst others. The neuropathological abnormalities included cerebral and/or cerebellar herniation (16%), cerebral edema (13.8%), subarachnoid hemorrhage (10%), recent cerebral infarction (9%), intraparenchymatous hemorrhage (8.14%), hypoxic-ischemic encephalopathy (3%) and other events such as Sneddon syndrome and Lupus vasculitis. These findings express that the cerebrovascular phenomena in this age group are unique and closely related with the underlying disease.     

Epilepsy in middle-aged and elderly people: a three-year observation.

An analysis of the medical documentation and investigation of 130 cases of epilepsy diagnosed in a group of people over 50 years of age (average: 65.4 years) revealed that the most common type of seizure in the group studied was partial (66.2%), followed by seizures with secondary generalization (33.8%). Epilepsy was caused by cerebrovascular disease (50.8%) considerably more often in patients over 74 years of age, craniocerebral trauma in patients addicted to alcohol (13.1%), especially those under 65 years of age, primary or metastatic neoplastic disease (10.7%), and others. The authors wish to draw attention to the leukoaraiosis factor, which might be the proepileptogenic cause of epilepsy recognized in the group of patients over 74 years of age (56.5%) and is much more frequent in this group than in the group of patients under 65 years of age (1.6%). Moreover, some drugs, such as L-dopa and Baclofen, might have been related to the epileptic seizures. In 29 patients (22.3%) the definite cause of late-onset epilepsy was unknown. The authors suggest in such cases, both follow-up tomographic examination and careful clinical examinations. In the study group of patients with initially unknown seizure etiology, some diseases, such as cerebral tumor or colon and pancreatic neoplasm, were diagnosed during follow-up examination. These processes were revealed several months after the first epileptic seizure.     

Health status and ADL functioning of older persons with intellectual disability: community residence versus residential care centers

The objective of the study was to study differences in aging phenomena among adults with intellectual disability (ID), who live in community residence versus their peers in residential care centers and to determine the contribution of health status, age, gender, etiology and level of ID to the decline in ADL function with age. Our study was based on matched pairs between persons with ID in community residence (N=101) and their peers living in residential centers (N=101) by age, gender, etiology and level of ID. Fifty-three percent were aged 40-49 years, 23% were aged 50-59 years and 14% were aged 60-71 years. Ten percent had Down syndrome (DS), 16% had cerebral palsy (CP) and the rest had ID with no specific etiology (NSID). Caregivers were interviewed to ascertain health problems, sensory impairments and activity of daily living (ADL). The type of residence alone could not explain the morbidity and health problems of adults with ID, which were affected by age and etiology. Participants with NSID functioned better than those with Down syndrome or cerebral palsy in all ADL areas. MANOVA and regression analysis indicated that age and health status did not contribute to the explained variance of the ADL function of the participants without specific etiology. The CP group had the most vulnerable etiology exposed to medical problems and decline in ADL function with age. Action should be taken to increase the awareness of the staff to health deterioration that can occur among adult persons with ID, especially among various types of etiologies, such as DS and CP.     

Comparison of secondary vascular prevention in practice after cerebral ischemia and coronary heart disease

BACKGROUND: Secondary prevention measures in patients with cerebral ischemia or coronary heart disease (CHD) consist of vascular risk factors management and antithrombotic therapy. The aim of this study was to compare how secondary prevention measures are applied in practice between patients with a history of CHD or cerebral ischemia. METHOD: We compared vascular risk factors management and antithrombotic therapy between patients with a history of CHD and patients with a history of cerebral ischemia that occurred 2 months to 6 years earlier. Whether patients were properly treated or not was determined by a comparison between their current treatments and European guidelines for stroke and CHD management. RESULTS: We included 107 consecutive patients with a history of cerebral ischemia and 85 consecutive patients with a history of CHD. We found that: (i). most patients did not receive an appropriate secondary prevention in both groups: 76 of 107 patients with previous cerebral ischemia (71 %) and 73 of 85 patients with CHD (85.9 %); (ii). identification of risk factors, such as hypercholesterolemia, diabetes mellitus and smoking, did not differ between both groups, but arterial hypertension was more frequent in CHD patients; (iii). an inappropriate management of risk factors was more frequent in patients with TIA vs. ischemic stroke, and angina pectoris vs. myocardial infarction; (iv). arterial hypertension and hypercholesterolemia were the 2 more frequent risk factors that were not properly treated; (v). more than half diabetic patients had hyperglycemia > or= 126 mg/dl in both groups; (vi). patients with previous CHD had twice more frequently stopped smoking than those with cerebral ischemia. CONCLUSION: Many patients were not properly treated in both groups, and differences between practice and guidelines were more frequent in the CHD group, where guidelines are more strict.     

Acute encephalitis

Acute encephalitis: etiology, clinical findings and prognosis. We studied 44 patients with acute encephalitis diagnosed in a neurological university clinic during an 11-year period. An etiology was found in 11 cases (25%). In 3 the cause was herpes simplex virus; in 2 morbilli. There were single patients in which the cause was mycoplasma pneumoniae, epidemic parotitis, ornithosis, infectious mononucleosis, influenza B-virus and recent tetanus immunization. No specific etiology was found in 33 (75%). Besides fever the most frequent sign was impairment of consciousness in 39% of cases. Four patients (9%) died. Among the survivors mental and/or focal neurological deficits persisted in 22 (55%). Most frequent was dementia in 6 cases (15% of survivors). Impaired consciousness in the acute phase indicated a worse prognosis (p < 0.005).     

Dissection of the brain supplying arteries over the life span

Cervical artery dissection (CAD) is being increasingly diagnosed due to improved neuroimaging methods. The mean age of patients with CAD is about 40 years, with a peak between 40 and 45 years of age. Proven data on the incidence of CAD in older patients are missing. Therefore, whether CAD should also be considered as a probable cause of cerebral ischemic events in the elderly was investigated. All consecutive patients referred to our clinic with a diagnosis of cerebral ischemia from January 1999 until June 2000 were thoroughly assessed for the presence of CAD. In addition, the records of all stroke patients treated in our department from January 1995 to December 1998 were analysed retrospectively for a diagnosis of CAD. A total of 34 patients (11 women) had suffered from internal carotid (n = 29) or vertebral artery (n = 5) dissection. Their mean (SD) age was 50.3 (14.6) years, with 32.4% being older than 60 years. Clinical presentation of CAD did not differ depending on the patients' age. The results show that CAD is a possible cause of cerebral ischemia in the elderly and thus has also to be considered in the diagnostic investigation in this patient group.     

Symptomatic dystonia: clinical profile of 46 Brazilian patients.

Dystonia is a syndrome characterized by sustained muscle contraction, provoking twisting and repetitive movements or abnormal postures. It may be classified according to etiology, as idiopathic or symptomatic. We studied 122 Brazilian patients with a dystonic syndrome. Of these, 46 (37.7%) had symptomatic dystonia. The most frequent cause was tardive dystonia (34.8%) followed by perinatal cerebral injury (30.4%). Other causes were stroke (13.0%), encephalitis (6.5%) and Wilson's disease (4.3%). Cranial trauma, mitochondrial cytopathy and psychogenic, were the least frequent causes with one patient in each category. The etiology in two patients could not be established. Perinatal cerebral injury and postencephalitic dystonia were seen in the younger age group, while post-stroke and tardive dystonia were seen in the older age group.     

A case of postpartum cerebral angiopathy with intracranial hemorrhage and subarachnoid hemorrhage immediately after delivery]

A 32-year-old woman, gravida 0, para 0, was admitted for delivery at 40 weeks of gestation. She had no history of headache, hypertension, and toxemia. She was delivered of a healthy boy. Although she was given no medication during normal delivery, she suddenly became drowsy and developed left hemiparesis immediately after delivery. Computed tomographic (CT) scan of the brain performed on the admission day revealed a subarachnoid hemorrhage and right putaminal hemorrhage. A 4-vessel cerebral angiogram demonstrated multiple irregular narrowing of the anterior cerebral arteries, middle cerebral arteries, and posterior cerebral arteries. Her conditions seemed to improve without any medication, and the multiple stenoses were no longer observed by angiography in a follow-up examination. On the basis of these observations, she was diagnosed as having postpartum cerebral angiopathy (PCA). Pathogenesis of PCA still remaing unclear to date an ergot alkaloid derivate (ergonovine) and the hydrogenated form of the powerful vasoconstrictor ergot (bromocriptine) have been reported as cause of PCA. We will classify PCA into 3 categories: eclampsia, secondary PCA, and primary PCA. "Primary PCA" is of unknown etiology, "secondary PCA" results from a known etiology such as drugs, and "eclampsia" is a PCA with toxemia of pregnancy. We report here, that PCA may occur even in a normal pregnant woman who was given no medication, and should be considered in the diagnosis of postpartum cerebral vascular diseases in women.     

Etiology of and risk factors for cerebral infarction in young adults in western Norway: a population-based case-control study

We sought to study the etiology of and risk factors for cerebral infarction in young adults in Hordaland County, Norway. All patients aged 15-49 years living in Hordaland County with a first-ever cerebral infarction during 1988-97 were included. Etiology was analyzed in subgroups defined by sex, age (<40 years versus >/=40 years), circulation territory (anterior versus posterior circulation) and short-term functional outcome [modified Rankin score (mRS) 2]. A questionnaire was used to evaluate possible risk factors amongst the patients compared with an age- and sex-matched control group. The distribution of etiology was significantly different in all subgroups. Atherosclerosis was frequent amongst men (22.8% vs. 4.2%) and patients >/= 40 years (20.8% vs. 2.7%). All patients with microangiopathy had favorable short-term outcome. Significant risk factors were smoking more than 15 cigarettes per day (P < 0.001), hypertension (P = 0.001), and myocardial infarction (P = 0.035). Modifiable risk factors were frequent.     

Non-Traumatic Intracerebral Hematoma in the Young: Findings from a Tertiary Hospital in the Philippines

BackgroundThere is geographic variability in the clinical profile and outcomes of non-traumatic intracerebral hematoma (ICH) in the young, and data for the Philippines is lacking. We aimed to describe this in a cohort from the Philippines, and identify predictors of mortality.MethodsWe performed a retrospective study of all patients aged 19–49 years with radiographic evidence of non-traumatic ICH admitted in our institution over five years. Data on demographics, risk factors, imaging, etiologies, surgical management, in-hospital mortality, and discharge functional outcomes were collected. Multivariate logistic regression analysis was done to determine factors predictive of mortality.ResultsA total of 185 patients were included, which had a mean age of 40.98 years and a male predilection (71.9%). The most common hematoma location was subcortical, but it was lobar for the subgroup of patients aged 19-29 years. Overall, the most common etiology was hypertension (73.0%), especially in patients aged 40-49. Conversely, the incidence of vascular lesions and thrombocytopenia was higher in patients aged 19-29. Surgery was done in 7.0% of patients. The rates of mortality and favorable functional outcome at discharge were 8.7% and 35.1%, respectively. Younger age (p = 0.004), higher NIHSS score on admission (p=0.01), higher capillary blood glucose on admission (p=0.02), and intraventricular extension of hematoma (p = 0.01) predicted mortality.ConclusionsIn the Philippines, the most common etiology of ICH in young patients was hypertension, while aneurysms and AVM's were the most common etiology in the subgroup aged 19 – 29 years. Independent predictors of mortality were identified.     

A study of cerebrovascular autoregulation and CO2 reactivity in putaminal hemorrhage

Regional cerebral blood flow (rCBF) during hypertension and hypercapnia was studied in 33 patients with putaminal hemorrhage, using a single photon emission CT by means of Xenon 133 inhalation method. The results obtained were as follows: 1) A significant relationship was obtained between the impairment of autoregulation, CO2 reactivity and the degree of cerebral ischemia, i. e., in most cases, these vascular responses were impaired in cases of ischemia showing the rCBF decrease over 30 to 40% of normal values. However, there were particular cases with cerebral ischemia of over 30 to 40% in which autoregulation seemed to be preserved in the acute stage, which was considered to be the similar phenomenon as so called "false autoregulation". 2) The cerebrovascular responses such as autoregulation and CO2 reactivity were preserved in cases of less than 50 ml of hematoma volume. In cases with 50 to 74 ml of hematoma volume however, autoregulation and CO2 reactivity were mostly impaired, especially in the affected hemisphere rather than the non-affected, in the period of 1 to 2 months from the onset. Furthermore, the impairment was also involved in both hemispheres if the hematoma was over 75 ml in volume. 3) The cerebrovascular responses were markedly impaired in the region of basal ganglia of the affected hemisphere which corresponded well to the hematoma site. 4) There was a close correlation between the cerebrovascular responses and the activity of daily life (ADL), i. e, the prognosis might be poor in cases with global impairment, but which seemed to be rather good in cases with local impairment. It might be concluded, from the results mentioned above, that the study of autoregulation and CO2 reactivity is probably significant in estimating the pathogenesis and the treatment of cerebral ischemia following hypertensive putaminal hemorrhage.     

Detection of cerebral artery fenestrations by computed tomography angiography

Background and purposeCerebral artery fenestrations (CAF) are rare congenital variations usually diagnosed by digital subtraction angiography (DSA). The aim of this study was to examine the frequency of occurrence of fenestrations in cerebral arteries and their coexistence with cerebral aneurysms in computed tomography angiography (CTA).Material and methodsAll reports of cerebral CTA (1140) performed in one institution from March 2005 to December 2007 were analysed. We found 40 patients with single fenestrations of the intracranial arteries. All 40 examinations were retrospectively reviewed for location of vascular malformations and presence of aneurysms or subarachnoid haemorrhage (SAH). Medical histories of those patients were then analysed for evidence of SAH and referral reasons for CTA.ResultsForty fenestrated arteries were found in CTA: 18 basilar arteries (45%), 16 anterior cerebral arteries (40%), 4 anterior communicating arteries (10%) and one middle cerebral artery (2.5%). Only one vertebral artery fenestration was found due to the technique of the examination. Six patients (15%) with fenestrated arteries had a total of 8 aneurysms, although only one aneurysm was ipsilateral to the fenestration. In 8 cases of SAH, two were with no evidence of vascular malformation. The coexistence of CAF and aneurysms in CTA amounted to 15% (6/40), but the incidence of ipsilateral aneurysm was only 2.5% (1/40) and it affected the anterior cerebral artery.ConclusionsBasilar artery fenestration is the most frequent observed fenestration in CTA, followed by anterior cerebral artery and anterior communicating artery fenestrations. Coexistence of fenestration and aneurysm is uncommon in CTA examination.     

Toxic myopathies: muscle biopsy features

Several drugs and toxic substances can cause muscular abnormalities and are frequent causes of acquired myopathies. We present a series of 32 patients, predominance of young adult patients, diagnosed with toxic myopathy. The most common substances inducing myopathy were corticosteroids (56.2%) followed by the propoxyphene, neuroleptics, zidovudine and drug-induced hypokalemia. The investigation showed normal serum creatine kinase levels in 65.4%, myopathic pattern of the needle electromyography in 40% and the more frequent histological diagnosis of the muscle biopsy was type 2 fiber atrophy (59.3%). Clinical features, etiology, course of the disease, serum levels of muscular enzymes, electromyographic features and, especially, muscle biopsy features are discussed.     

Antiphospholipid antibodies and cerebral ischemia in young people

The importance of a prothrombotic state as a cause of ischemic stroke in young adults is ill defined. We examined 46 unselected patients under age 50 years with cerebral ischemia for anticardiolipin antibody (aCL) and lupus anticoagulants (LA), over a 3-year-period. Age- and sex-matched patients with other neurologic diseases served as a noncerebral ischemia comparison group to test whether (1) stroke/transient ischemic attacks (TIA) in young people is associated with aCL and/or LA, and (2) their presence is specific to cerebral ischemia. In the stroke/TIA group, 21 patients had aCL or LA and 25 had neither, whereas in the control group, 2 patients had aCL and 24 had neither. Equal numbers of stroke/TIA patients with and without antiphospholipid antibodies (aPL) had other stroke risk factors. Patients with aPL and cerebral ischemia, however, had a more frequent history of multiple events than those without them. These antibodies occur with undue frequency in young patients with stroke/TIA and are not associated with a concurrent diagnosis of systemic lupus in most cases. A coexistent aPL-associated prothrombotic state may be a key determinant of whether patients with atherosclerosis, mitral valve prolapse, or other structural lesions experience recurrent ischemia.     

Acute hemisyndrome in childhood (author's transl)

Neurologic hemisyndromes in childhood may be due to congenital or acquired changes in cerebral vessels. Among the congenital vascular malformations, which very rarely become manifest already in children, the arteriovenous angiomata and saccular aneurysms are relatively the most common (in childhood about 4-8% manifest, 1-2% of all diseases). Venous angiomata, micro-angiomata and congenital dissecting aneurysms are very rare. But acquired thrombotic blocks of cerebral arteries are of greater importance as causes of acute neurologic hemisyndromes in childhood. Most often they have an inflammatory or traumatic cause, often the cause is unknown. Rare diseases only very recently described as arterial blocks of cerebral vessels are fibromuscular dysplasia and Moya-Moya-disease, the etiology of which is not yet fully understood. The prognosis of these diseases is generally unfavorable, but the focal signs in migraine as visual, sensory, aphasic and motoric defector irritative signs are as a rule reversible. In order to establish, where possible, the cause of acute neurologic hemisyndromes in childhood, early angiography may-classic migraine accompagnee types excepted-help. But also modern biochemical, immunologic, virologic, serologic, bacteriologic, clotting analytic and, possibly, histologic and histochemical techniques should be employed with particular attention to fat metabolism and to auto-immune disease.     

Jitteriness beyond the neonatal period: a benign pattern of movement in infancy.

Jitteriness is a frequent, well-recognized phenomenon in neonates. Its occurrence in early infancy, beyond the neonatal period, is much less documented. Thirty-eight full-term infants who were jittery after 6 weeks of age were followed until the jitteriness disappeared and then reevaluated at the age of 3 years. The jittery movements had already been observed during the neonatal period in 16 babies but not before 6 weeks of age in 22. In 34 infants (89%), 1- and 5-minute Apgar scores were 9 or 10. At the initial examination, a mildly increased muscle tone was found in 12 infants, and hyperactive deep-tendon reflexes were found in 19. The jitteriness resolved at a mean age of 7.2 +/- 3.4 months. At 3 years, 92% of the infants had a normal neurodevelopmental examination, while in the rest only minor, transient disturbances were found. This study indicates that jitteriness as a sole presentation in infants beyond the neonatal period is a benign phenomenon, associated with an excellent prognosis. Its etiology is unknown but seems most likely to be related to a maturational process within the central nervous system.     

Etiopathogenesis and prognosis of cerebral ischemia in young adults.

Etiology and long-term prognosis were prospectively investigated in 155 consecutive patients (96 men and 59 women), aged 16 to 45 years, referred to our Neurosurgical Unit with cerebral transient ischemic attacks or infarction during the period 1978-1988. All patients underwent neurological and medical-cardiological evaluation, cerebral computerized tomography scanning, electrocardiogram, and laboratory tests. Two-dimensional echocardiography was performed in 123 cases (79%), cerebral angiography in 147 (95%). Atherosclerosis was the leading etiology occurring in 48 patients (31%). A cardioembolic disorder was considered the probable cause of ischemia in 8 cases (5.1%). Further possible etiologies were contraceptive pill assumption (5.8% of the total, but 15.3% within the female group), spontaneous arterial dissection (4.5%), migraine (4%), puerperium (2.6%), cervical trauma (2.6%), and other, more uncommon conditions. Despite extensive evaluation, the cause of cerebral ischemia remained unknown in 40% of cases. All patients received antiplatelet medication and 16 underwent surgery. The long-term outcome at a mean follow-up of 5.8 years was favorable: 91% of subjects resumed their work on a full or part-time basis.     

Intracerebral hemorrhage in young people: analysis of risk factors, location, causes, and prognosis

BACKGROUND AND PURPOSE: The frequency of intracerebral hemorrhages (ICHs) in people aged 31 years (odds ratio, 3.48), and those with ICH that resulted from arteriovenous malformations were aged <20 years (odds ratio, 2.80). The final outcome was considered favorable in 60%. CONCLUSIONS: ICHs in young people are mainly lobar in location and result from vascular malformation. Hypertension causes most cases in which the ICH is located in the basal ganglia. Mortality and morbidity in the acute phase are low and are related to hypertension as the cause of ICH.     

West syndrome--The University of Hong Kong experience (1970-2000).

OBJECTIVE: To study the clinical pattern of West syndrome (WS) in a university based hospital. METHODOLOGY: The database of children seen in the Epilepsy Clinic of Queen Mary Hospital and Duchess of Kent Children's Hospital during a 30-year period (1970-2000) was reviewed. RESULTS: A total of 105 cases had WS (1.9%). The number of new cases of WS admitted per year ranged from one to eight. The range of annual incidence of WS to newly diagnosed epilepsy was 0.8-4.8%. The etiology included idiopathic (N=19, 18%), cryptogenic (N=23; 22%), symptomatic (N=56; 53.3%) and unknown (N=7; 5.7%). Adrenocorticotropic hormone (ACTH) and/or prednisone were given to 42 children (40%). Most were effective in controlling WS on an all-or-none fashion. Seizure outcome included 12 with remission, persistent in the same form in two and persisting but changed to another form in the rest. Neurological outcome at the last follow up in 2000 December included multiple disabilities (N=16), cerebral palsy (N=22), mental retardation (N=94) and Lennox-Gastaut syndrome (N=13). We analysed the following risk factors in relation to poor outcome: age of onset, age of presentation, time lag before treatment, etiology, family history of epilepsy and hormonal treatment. Only etiology of WS has a positive correlation with poor outcome (P<0.0005). CONCLUSIONS: WS is an uncommon epileptic syndrome. The majority had poor outcome, especially those with causes identified. Infantile spasm is a specific epileptic phenomenon in a maturational stage of a child when heterogeneous disorders can present with the same clinical epileptic and electroencephalographic phenomenon.