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The association between migraine and juvenile stroke: a case-control study.

BACKGROUND: Several studies suggest an association between migraine and juvenile stroke. Because of some shortcomings, we designed another case-control study of a homogenous group of patients with juvenile cerebral ischemia. This study is part of a larger German epidemiological research project on the association of migraine with cerebrovascular disease. METHODS: We enrolled 160 consecutive patients under the age of 46 years with first-ever ischemic stroke or transient ischemic attack and 160 strictly sex- and age-matched controls. Patients suffering from arterial dissection, brain hemorrhage, cranial sinus thrombosis, lacunar stroke, or from migrainous infarction were excluded. Migraine was diagnosed according to the criteria of the International Headache Society by the same 2 independent interviewers. For analyzing the data, nonparametric statistical methods including odds ratio and 95% confidence interval were used. RESULTS: Migraine was a significant risk factor for juvenile stroke for the total sample with an odds ratio of 2.11 (confidence interval, 1.16 to 3.82). The odds ratio was even higher in the subgroup under the age of 35 (3.26) and in the female subgroup (2.68). We found migraine to be independent from other vascular risk factors, from etiology, and from the territory of stroke. CONCLUSION: We can confirm the findings of previous studies showing a significant association between migraine and juvenile stroke in women. Furthermore, our data suggest migraine to be an even more significant risk factor for patients under the age of 35 and to be independent from other vascular risk factors.

Adolescent↗

Magnetic resonance demonstration in the newborn of generalized cerebral venous dilation with spontaneous resolution.

The authors present serial magnetic resonance (MR) images of an infant with cardiac failure and generalized cerebral venous dilation, which was initially misdiagnosed in the first week of life on cranial ultrasound as a vein of Galen malformation. At 3 months of age, repeat MR imaging demonstrated complete resolution of this marked cerebral venous distension with no evidence for cerebral injury. This case illustrates the value of MR in the identification of this disorder and its distinction from more serious conditions, such as vein of Galen malformation and venous sinus thrombosis. Complete resolution of the venous dilation and the lack of definable parenchymal injury suggest a good prognosis for this disorder.

Cerebral Veins↗

[Therapy of acute mastoiditis].

BACKGROUND: Acute otitis media is a common disease, mostly contracted at childhood. The development of acute mastoiditis has been decreased since the introduction of antibiotics. Currently it is only developed in 0.004 % cases of acute otitis media. But despite of this fact, even today one should not neglect this condition as the complications such as intracranial spread and lateral sinus thrombosis are still life threatening. METHODS AND PATIENTS: In a retrospective study from 01/96 - 09/00 we reviewed the cases of acute mastoiditis as a result of acute otitis media at our department. Additionally we compared study results out of 1972 until 1989 with our current results. RESULTS: Altogether mastoidectomy has been carried out at 157 patients. The cases have steadily increased since 1996. This is confirmed by comparison with study results of previous years. The majority of the patients were between 8 months and 3 years old (60 %). When the patients first presented at our department, the first signs of otitis media were 19.5 days ago in average. 38 % of the patients received no antibiotic treatment before admission. A thickened mucous membrane was seen by the mastoidectomy mostly (58 %). A subperiostal abscess we noted in 8.5 %. The most common isolated bacteria were Streptococcus pneumoniae (33 %) and Streptococcus pyogenes (11 %). The patients stayed at the department about 10 days. In comparison to recent study results the "typical" mastoiditis has decreased. The causative pathogens have not changed. CONCLUSIONS: In the last years we realised an increasing number of mastoiditis at our department. The isolated bacteria are the same as presented in the literature. Early surgery combined with an effective antibiotic treatment can avoid the known complications of mastoiditis.

Acute Disease↗

MRI findings in pediatric neuro-Behçet's disease.

Behçet's disease (BD), a systemic vasculitis of unknown cause, affects many organs and systems. Neurological involvement is seen in 5-15% of the patients, and the two major forms of neurological disease seen in BD are central nervous system (CNS) parenchymal involvement and cerebral venous sinus thrombosis. We report a 14-year-old boy with BD who had neuro-parenchymal involvement. The diagnosis of the systemic disease was not made until the onset of the neurological manifestations, which led to an MRI study that revealed findings suggestive of CNS involvement of BD. We therefore emphasize the importance of the localization and appearance of other characteristics of the lesions on MRI in the differential diagnosis of parenchymal neuro-Behçet syndrome.

Adolescent↗

[Acute mastoiditis in children, a retrospective analysis about a period of 25 years].

BACKGROUND: During the last years the classical signs of the acute mastoiditis have changed. Aim of the following study was to examine the change of symptoms as well as the change of incidence. METHODS/PATIENTS: The records of 113 children who received mastoid surgery due to acute mastoiditis were retrospectively investigated. Data covered a regional group of patients during a period of 25 years (1979 - 2003). RESULTS: Considering the drop of the birthrate from 1991 in the East German countries, there was a significant increase of the incidence of acute mastoiditis. The patients had a mean age of 2,5 years. The classical symptoms like "retroauricular swelling, tenderness and protrusion of the auricle" were only noted by 47 children (41.6 %). Preoperative complications appeared as facial palsy, abscess and septic lateral sinus thrombosis in 11 children. Bacterial cultures, taken during surgery yielded in 55.7 % Streptococcus pneumoniae. The therapy contained a surgical intervention combined with antibiotics. CONCLUSIONS: An early surgical treatment by mastoidectomy or antrotomy supported by antibiotics represents the safest method to avoid complications.

Acute Disease↗

[Cerebral ischemia in young adults].

An overview is given over etiology and prognosis of cerebral ischemias until the age of 40. In a time period of 19 years, 168 patients were diagnosed with cerebral ischemia until the age of 40 (91 females, 77 males). The most frequent etiology is premature atherosclerosis in patients with vascular risk factors (up to 50%). Cardiogenic embolism is responsible for 1 to 34% of the cases: cardiac valve diseases and endocarditis being the most frequent sources. In 2 to 19% a vasculitis is diagnosed. While infectious arteritis is especially frequent in countries of the third world, immunovasculitides are common in Europe and the USA. Noninflammatory vasculopathies include spontaneous or traumatic dissection, fibromuscular dysplasia and vascular malformations. A migrainous stroke is especially frequent in female smokers with intake of oral contraceptives. During pregnancy both sinus thrombosis and arterial ischemia occur. Hematologic causes for ischemia are polycythemia, thrombocytosis and genetic diseases (sickle cell anemia, AT3-deficiency). Cerebral ischemia may occur in connection with the ingestion of ergot-derivates. The prognosis of cerebral ischemia in young adults is better than in older stroke-patients.

Adolescent↗

[Isolated unilateral otitis with facial nerve paralysis as initial symptom of Wegener granulomatosis. An unusual clinical course].

BACKGROUND: Wegener's granulomatosis is an immunepathogenic disease of unknown origin. The histopathological picture shows granulomatous inflammation with epitheloid cells, granulomas, and general vasculitis. The diagnosis of Wegener's granulomatosis is made on the basis of the clinical picture, serum cANCA, and histologic examination of biopsies. PATIENT: We present the case of a 57-year-old white male patient who was admitted to our ENT Hospital with a six weeks' history of otalgia and incomplete ipsilateral facial palsy since the day before admission. The patient had clinical features of acute otitis media without signs of mastoiditis. Despite a ten days' course of intravenous antibiotic treatment, the intensity of facial palsy progressed and the general condition of the patient worsened. A mastoidectomy and decompression of the facial nerve were performed, demonstrating sclerosis of the mastoid cells. Three weeks after release from the hospital, the patient was admitted again with recurrent fever, cephalea, loss of weight, and arthritic pain. There were no signs of recurrent otitis media or mastoiditis, and sigmoid sinus thrombosis was ruled out. Even under aggressive, intravenous antibiotic treatment the general physical condition continued to worsen; septic temperatures and signs of beginning renal failure occurred. The patient was transferred to the ICU with the diagnosis of sepsis of unknown origin. There bloodtests were positive for cANCA, which is highly specific for Wegener's granulomatosis. Under therapy with cyclophosphamide and i.v. corticosteroid, the patient recovered with 14 days. CONCLUSION: The lack of symptoms in the upper respiratory tract in our patient was unusual, indicating that in patients with recurrent otitis media, facial palsy, mastoiditis, or external otitis Wegener's granulomatosis should be ruled out as differential diagnosis.

Antibodies, Antineutrophil Cytoplasmic↗

[MR angiography in neuropediatric problems: the technic and the clinical results].

31 children aged between 6 months and 14 years with various neurovascular abnormalities were examined by MR angiography in a prospective study. In order to test the sensitivity and specificity of the MR results, these were compared in 8 cases with the findings of digital subtraction angiography or conventional angiography. In 24 patients arterial MR angiography was performed; in 17 these showed anomalies or abnormalities of the intracranial vascular system. In 7 patients venous MR angiography was performed and demonstrated the presence of sinus thrombosis in 6 of these. Comparison between MR angiography and DSA showed agreement in 7 cases; in one patient the degree of a stenosis was exaggerated by MR. The results of this study indicate that arterial and venous MR is of great value as an additional study in paediatric neurovascular problems.

Angiography, Digital Subtraction↗

[Reversible bilateral changes in cerebral proton-spin tomography in eclampsia].

HISTORY AND CLINICAL FINDINGS: Case 1: A 19-year-old primigravida was hospitalised in the 24th week of pregnancy after a series of grand-mal seizures. Investigation showed the classical eclamptic triad of oedema, hypertension and proteinuria. Case 2: 6 days post-partum a 24-year-old primipara developed hypertension and typical clinical prodromata. Subsequently she had a series of sometimes initially focal grand-mal seizures. TREATMENT AND COURSE: In both cases seizures ceased following pharmacological treatment. The pregnancy in the first case remained intact. Cerebral magnetic resonance imaging (MRI) demonstrated characteristic findings in both cases, in case 1 bilateral disseminated supratentorial foci of oedema without space-occupying features, in case 2 occipital white matter oedema and several supratentorial foci. All changes were completely reversible, in case 1 after 10 days, in case 2 after 16 days. CONCLUSIONS: Together with the clinical findings the focal changes on MRI provide a characteristic picture. MRI can thus be an important aid to the diagnosis of eclampsia and will prevent a misinterpretation as focal encephalitis or venous sinus thrombosis.

Adult↗

[Ptosis in the differential diagnosis of neurologic diseases].

This review summarizes those neurological diseases which are accompanied by a drooping of the upper lid, due to weakness of the m. levator palpebrae or m. tarsalis respectively. After connatal ptosis with or without involvement of other bulbar muscles the different types of muscular dystrophies are mentioned. Myositis, disturbances of potassium regulation and myasthenia gravis are other causes of ptosis. Diseases involving the oculomotor nerve (aneurysm, upper herniation, cavernous sinus thrombosis, orbital cavity processes, superior orbital fissure syndrome) may lead to associated ptosis. Metabolic disturbances, such as diabetes mellitus, Wernicke's encephalopathy and botulism may be accompanied by ptosis. Infectious diseases such as polyneuritis, meningitis or encephalitis can lead to ptosis. Sympatholytic ptosis is due to diseases of the central or peripheral course of the sympathetic nerve from the diencephalon via the cervical medulla, the neck, internal carotid artery to the superior orbital fissure. This type of ptosis is usually accompanied by miosis and often by sweating loss on the same side.

Blepharoptosis↗

[Acquired protein C deficiency in ulcerative colitis. The cause of thromboembolic complications].

Three patients with an acute exacerbation of ulcerative colitis (a 40-year-old and a 31-year-old man and a 30-year-old woman) developed a protein C deficiency (serum protein C activity between 32 and 48%). In the two men the protein C deficiency was diagnosed only after the onset of severe thromboembolic complications (cavernous sinus thrombosis; pulmonary embolism) during heparin treatment. But in the woman protein C activity was measured immediately after hospital admission (in the knowledge of the first two cases) even before heparin administration was started. All three patients received treatment with sulphasalazine (3 g daily) and fluocortolone (60 mg daily), as well as full heparinization (22,500-36,000 IU daily). Protein C activity returned to normal on remission of the ulcerative colitis (in one case only after subtotal colectomy). These case reports show that acquired protein C deficiency can be reversed by rigorous treatment of the underlying disease.

Adult↗

Neurological complications in children with protein C deficiency.

Four children with a cerebrovascular occlusive accident and protein C deficiency are described. Two patients presented with an acute hemiplegia, the others suffered from a transient ischemic attack and a progressive hydrocephalus as the result of sinus thrombosis. In all cases protein C deficiency, Type 1, was diagnosed. Other causes of cerebrovascular disease were excluded. Although venous thrombosis has been extensively reported in protein C deficiency, these cases indicate that protein C deficiency is also related to arterial thrombosis. In evaluating children with cerebrovascular accidents, protein C deficiency should also be considered.

Adult↗

Fetal arachnoid cysts: their site, progress, prognosis and differential diagnosis.

Arachnoid cysts are lined by arachnoid membranes and filled with cerebrospinal fluid. Etiologically, they are thought to be due to maldevelopment of the arachnoid or secondary to trauma or infection. Postnatally, many are asymptomatic and remain quiescent for years, although others expand and cause symptoms by compressing adjacent brain and/or expanding the overlying skull. Being congenital, it should be possible to identify them in the fetus, and this has proved to be so. But are they anatomically similar and do they behave the same as those detected postnatally? Fifteen fetuses with fluid-filled cysts were identified from the database of the Fetal Management Unit at St. Mary's Hospital in Manchester. Five were diagnosed at or before 20 weeks of gestation, 4 between 21 and 30 weeks, and 6 at 31 weeks or more. Thirteen cysts were in the supratentorial compartment, and 2 in the posterior fossa. Eleven fetuses were delivered, and 4 pregnancies were terminated. One of the born children had Pallister-Hall syndrome and died on day 19, and another had Aicardi's syndrome, is retarded and has fits, 1 with a posterior fossa cyst developed hydrocephalus in utero and was delivered early for the insertion of a ventriculoperitoneal shunt, he is moderately mentally delayed. Of the remaining 8 children, 1 has been lost to follow-up, and 7 appear to be reaching their early milestones on time. The 4 terminated fetuses had a postmortem examination, 2 did not have arachnoid cysts; 1 had an expanding glioependymal cyst which had destroyed most of the cerebral hemispheres, and the other had a sagittal sinus thrombosis with extensive cavitation of one cerebral hemisphere. The diagnosis of an arachnoid cyst in the fetus can be difficult and may be confused with other fluid-filled cysts.

Arachnoid Cysts↗

Leptospirosis in Istanbul, Turkey: a wide spectrum in clinical course and complications.

Patients with high fever and multiorgan involvement were investigated for the determination of frequency, clinical course and complications of leptospirosis in Istanbul. Leptospirosis was determined in 22 cases among the 35 hospitalized patients that were pre-diagnosed as leptospirosis according to 'Probable Leptospirosis Diagnosis and Follow-up' form. Among the leptospirosis cases 19 were male and 16 were military staff. Mean age was 35.6 y. Dark field examination (DFE), latex agglutination test (LAG), ELISA IgM, leptospirosis culture (LC) and microscopic agglutination test (MAT) were performed to confirm the diagnoses. The most frequent initial symptoms and findings were fever, fatigue, headache, nausea-vomiting and increased muscle sensitivity. Jaundice was noted only in 2 cases. A 74-y-old female patient died after the recurrence of the disease with severe rhabdomyolysis and pulmonary failure. Sagittal sinus thrombosis, perimyocarditis and chronic renal failure were major complications in another 3 patients. ELISA IgM, LC, DFE, LAG and MAT tests were positive in 68, 72, 82, 100 and 100% of the patients, respectively. As a conclusion, diagnosis of leptospirosis is usually overlooked. Clinical awareness, use of probable leptospirosis diagnosis forms and the application of different laboratory methods in the diagnosis of suspected cases may offer the chance to diagnose the leptospirosis accurately.

Adult↗

Angiography in non-traumatic brain haematoma. An analysis of 100 cases.

PURPOSE: The primary purpose of this project was to study the anatomical characteristics of intracerebral haematoma (ICH) in order to determine features that may negate the need for angiography in some patients. MATERIAL AND METHODS: The study was prospective and designed to investigate the underlying cause of non-traumatic ICH in 100 cases assessed by conventional angiography. Patients were excluded if there was a history of trauma or known pre-existing brain abnormality. All patients were examined with CT and angiography within 4 days of the ictus. RESULTS: Ruptured aneurysms or arteriovenous malformations (AVMs) were diagnosed on the initial angiogram in 49% of cases: 27 AVMs and 22 aneurysms. One case of superior sagittal sinus thrombosis was also detected. Vascular abnormalities were found most frequently in the under-40 age group and in cases in which subarachnoid haemorrhage, intraventricular haemorrhage or extracerebral haematoma accompanied the ICH. The temporal lobe was the most frequent anatomical location (37%). When a temporal lobe haematoma extended into the Sylvian fissure from the inferior pole of the temporal lobe or when it was associated with subarachnoid haemorrhage, structural abnormalities were found in over 90% of cases. CONCLUSIONS: There are groups of patients with ICH in whom the CT features are highly suggestive of AVM or aneurysm rupture. If the initial angiography is negative in these cases, careful follow up by repeat angiography and/or MR imaging is essential. However, potentially treatable abnormalities cannot be excluded with certainty by the distribution of the haematoma on CT alone, even if there is a history of pre-existing hypertension.

Adolescent↗

Plateau-wave phenomenon (I). Correlation between the appearance of plateau waves and CSF circulation in patients with intracranial hypertension.

Plateau waves, as described by Lundberg (1960), can often be observed in patients with increased intracranial pressure (ICP) resulting from brain tumours, benign intracranial hypertension and other causes. The clinical significance of the waves, however, is still debated. In this study, the ICP was recorded continuously, the size of the ventricular system was evaluated using computerized tomography (CT), the cerebrospinal fluid (CSF) flow was studied using isotope cisternography, and the absorptive capacity of the CSF was determined by measuring the conductance to CSF outflow, in 94 patients with increased ICP. All patients who received continuous ICP monitorings had a basic ICP level of more than 200 mmHg, and these patients were assigned to two groups on the basis of the presence or absence of the plateau waves in the ICP recordings: group I comprising 17 patients without plateau waves but with a high ICP resulting from subarachnoid haemorrhage or hypertensive intracerebral haemorrhage, within 5 days after the start of bleeding; and group II consisting of 77 patients with both plateau waves and a high ICP resulting from brain tumours, meningitis carcinomatosa, benign intracranial hypertension, superior sagittal sinus thrombosis and communicating hydrocephalus. The isotope cisternography and conductance to CSF outflow studies showed that there was neither a delay in CSF circulation nor an impairment of CSF absorption in the group 1 patients and these patients showed no ventricular dilatation on CT, whereas there was a marked sluggishness in the CSF flow and a severe defect in the CSF absorption capacities of the group II patients irrespective of the presence or absence of ventricular dilatation on CT. The present observations indicate that patients with a plateau-wave phenomenon have a marked impairment of CSF absorption and CSF flow. We suggest that the phenomenon is an important sign indicating an impairment of CSF absorption capacities.

Adult↗

Plateau-wave phenomenon (II). Occurrence of brain herniation in patients with and without plateau waves.

From 1980 to 1989, during intracranial pressure (ICP) recording, brain herniation (central transtentorial herniation associated with uncal herniation) developed in 15 patients as a result of aneurysmal rebleeding, uncontrollable brain swelling and/or oedema in spite of attempts at treating increased ICP. The relationship between the ICP level at which herniation occurred and the ICP fluctuation patterns in continuous ICP recordings prior to the development of the herniation was studied retrospectively. As for the ICP fluctuation patterns, the patients were assigned to 2 groups on the basis of the presence or abscence of plateau waves of Lundberg: group I comprising 8 patients without plateau waves but with a high ICP, and group II comprising 7 patients with both plateau waves and a high ICP. In the group I patients the herniation occurred as a result of aneurysmal rebleeding or brain swelling after subarachnoid haemorrhage, and in the group II patients the herniation resulted from brain tumour, meningitis carcinomatosa and superior sagittal sinus thrombosis. In the group I patients the herniation developed at a mean ICP level of 70-98 mmHg, and in the group II patients it occurred at a mean ICP level of 120-150 mmHg, far higher than the ICP level causing herniation in the group I patients. It has been shown that patients with a plateau-wave phenomenon in continuous ICP recordings have a marked impairment of cerebrospinal fluid (CSF) absorption and a delayed CSF flow.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Clinical patterns of neurological involvement in Behçet's disease: evaluation of 200 patients. The Neuro-Behçet Study Group.

In order to define the patterns of neurological involvement in Behçet's disease and to assess prognostic factors, 558 files of the neuro-Behçet out-patient clinic were reviewed. Those patients without any evidence of objective neurological involvement as well as the patients with other possible explanations for the neurological picture, and cases not fulfilling the criteria for Behçet's disease were excluded. The remaining 200 cases (155 male, 45 female) were evaluated: 162 had parenchymal CNS involvement (brainstem or 'brainstem +' involvement in 51%, spinal cord involvement in 14%, hemispheric involvement in 15% and isolated pyramidal signs in 19%) while 38 had secondary or non-parenchymal CNS involvement. In the first group the most common findings were pyramidal signs, hemiparesis, behavioural changes and sphincter disturbance, whereas in the second group the syndrome of raised intracranial pressure due to dural sinus thrombosis was the main clinical manifestation. In 60% of the cases with parenchymal involvement, CSF was hypercellular and/or had an elevated protein level, whereas in cases with non-parenchymal involvement the CSF was usually normal except for the elevated pressure. In more than half of the patients with parenchymal involvement, MRI showed brainstem and/or basal ganglion lesions. Forty-one per cent of the cases had a course with at least one attack and remission, another 28% also had attack(s) but showed secondary progression, 10% had primary progression and 21% had silent neurological involvement. Survival analysis was performed in patients who had at least a 3-year duration of neurological disease. Parenchymal involvement, elevated protein and/or pleocytosis in the CSF, 'brainstem +' type involvement, primary or secondary progressive course and relapse during steroid tapering were all associated with a poorer prognosis.

Adult↗