Familial occurrence of migraine without aura and migraine with aura

We report a study of 121 probands (patients) with migraine without aura (MO) and 72 probands with migraine with aura (MA), diagnosed according to the operational diagnostic criteria of the International Headache Society and selected from 35 general practices in Denmark. The probands were interviewed about the presence of MO and MA among their first-degree relatives. Compared with the general population, the first-degree relatives of probands with MO had a threefold increase of MO, and only one first-degree relative of one proband with MO had MA. First-degree relatives of probands with MA had a twofold increase of both MA and MO. Compared with the general population, few spouses had MO and MA. This threefold and twofold increase in family risk of MO and MA, combined with the lack of increased risk in spouses, strongly suggests that MO and MA are genetically determined.     

Familial epilepsy with unilateral and bilateral malformations of cortical development

BACKGROUND AND OBJECTIVE: A variety of biologic or synthetic materials have been used in brow suspension ptosis surgery. We describe extrusion and granuloma formation in 5 cases of congenital ptosis operated with Mersilene mesh sling. PATIENTS AND METHODS: Case reports with review of literature. RESULTS: Two cases of extrusion and 3 cases of granuloma formation were encountered in 79 eye lids of 52 (6.3%) patients performed for the treatment of congenital ptosis. Mersilene mesh fibers in the cut surface and a foreign body reaction were found by microscopic evaluation of tissue harvested at the time of surgical removal of the sling. CONCLUSION: Despite the operative precautions, the probability of extrusion and granuloma formation should be in mind in cases of congenital ptosis operated with Mersilene mesh sling, a synthetic material.     

Familial cerebellar degeneration with slow eye-movements, mental deterioration and incidental nevus of ota (oculo-dermal melanocytosis)

In a bipartite rearing experiment (day 1-24 and 24-45) 72 early-weaned piglets were used to study the effect of varying dietary vitamin B6 contents on renal excretion of xanthurenic and kynurenic acid after a tryptophan load, on urea concentration and activities of two transaminases in serum at the end of each period. The animals, divided into 6 groups, were fed ad libitum a prestarter and a starter in period I and II, respectively, each containing 0.5, 1.2, 2.0, 2.8, 3.5 or 6.6 mg vitamin B6 per kg dry matter. The urinary xanthurenic acid excretion was elevated especially at the vitamin B6 supply of 0.5 ppm and rose severalfold with increasing depletion time (period II). In both periods, the smallest amount was excreted by piglets supplemented with 2.8 ppm. In comparison to groups B (1.2 ppm) and C (2.0 ppm), their average excretion rate was reduced by 29% and 15%, respectively, in period I and by 50% and 22%, respectively, in period II. Analogously to xanthurenic acid, the smallest amount of kynurenic acid was excreted by group D (2.8 ppm). Starting from the lowest vitamin B6 supply, the activity of SGPT showed an almost linear increase in both experimental periods. In contrast, SGOT already reached an upper activity level with the dietary vitamin B6 content of 3.5 and 2.8 ppm at the end of period I and II, respectively. The concentration of serum urea was influenced only by the lowest vitamin B6 supply of 0.5 ppm.     

An additional variant of the persistent primitive trigeminal artery: accessory meningeal artery--antero-superior cerebellar artery anastomosis associated with moyamoya disease

Neuropathy is a frequent complication in diabetes mellitus. Since the involvement of the autonomic nervous system indicates a poor prognosis, early detection and subsequent management are important. Analysis of heart rate variability (HRV) provides a quantitative measure of sympathovagal modulation activities on the heart and has been proven to be useful for the early assessment of the diabetic autonomic neuropathy. We recently developed a simple method of measuring pulse wave velocity (PWV) to evaluate sympathetic nervous activity in the vascular system. In this paper, we examined 33 diabetic patients with and without peripheral neuropathy (15 and 18 respectively) using these methods. In time domain analysis, the mean heart rate, standard deviation and coefficient of variation of HRVs significantly differed between these two groups, whereas the indices of PWVs did not show a significant difference. In frequency domain analysis of HRV, both low and high frequency components were decreased, and the low frequency component in normalized unit did not increase after standing in patients with peripheral neuropathy. We previously reported that the mean PWV decreased after standing in patients with diabetic neuropathy. This disagreement suggests that beta sympathetic dysfunction precedes alpha sympathetic dysfunction in diabetic neuropathy.     

Familial typical migraine: linkage to chromosome 19p13 and evidence for genetic heterogeneity

Migraine is a frequent familial disorder that, in common with most multifactorial disorders, has an unknown etiology. The authors identified several families with multiple individuals affected by typical migraine using a single set of diagnostic criteria and studied these families for cosegregation between the disorder and markers on chromosome 19, the location of a mutation that causes a rare form of familial hemiplegic migraine (FHM). One large tested family showed both cosegregation and significant allele sharing for markers situated within or adjacent to the FHM locus. Multipoint GENEHUNTER results indicated significant excess allele sharing across a 12.6-cM region containing the FHM Ca2+ channel gene, CACNL1A4 (maximum nonparametric linkage Z score = 6.64, p = 0.0026), with a maximum parametric lod score of 1.92 obtained for a (CAG)n triplet repeat polymorphism situated in exon 47 of this gene. The CAG expansion did not, however, appear to be the cause of migraine in this pedigree. Other tested families showed neither cosegregation nor excess allele sharing to chromosome 19 markers. HOMOG analysis indicated heterogeneity, generating a maximum HLOD score of 3.6. It was concluded that Chr19 mutations either in the CACNL1A4 gene or a closely linked gene are implicated in some pedigrees with familial typical migraine, and that the disorder is genetically heterogeneous.     

Magnetic resonance imaging of acute cerebellar ataxia: report of a case with gadolinium enhancement and review of the literature

INTRODUCTION: Intervention studies to reduce cigarette sales to minors have been conducted primarily in suburban settings. Little is known about sociocultural factors influencing cigarette sales to minors in urban settings. This study sought to determine sociodemographic and cultural factors that may play a role in cigarette sales and in efforts to reduce sales to minors in urban areas. METHODS: Merchant education and follow-up surveys were conducted in small local stores in predominantly African-American urban census tracts in Baltimore. The stores had prior evidence of cigarette sales to minors. RESULTS: Merchants reported hostility (66%) and foul language (64%) when they requested youth identification. Youthful-oriented advertising of cigarettes was highly prevalent in all stores and moreso in stores owned and staffed by Asian merchants. Advertising with specific youthful content was predictive (OR = 3.97; 95% CI = 1.70, 9.23; P = .0014) of higher requests for cigarettes from minors. CONCLUSIONS: Youth-oriented cigarette advertising is a prevalent environmental risk for urban youth. Differences between Asian and African-American merchants suggest socioethnic factors may be an influential component of illegal sales and educational campaigns to reduce smoking among minors.     

Primary meningeal malignant fibrous histiocytoma with cerebrospinal dissemination and pulmonary metastasis

Primary intracranial malignant fibrous histiocytoma (MFH) is very rare, and not much is known about its clinical features. The authors report a case of left temporal leptomeningeal MFH, with consequent cerebrospinal fluid (CSF) dissemination and pulmonary metastasis. The clinical features of this case and the therapeutic prognosis of 17 cases reported previously in the literature were reviewed.     

Exteroceptive suppression of temporalis muscle activity during migraine attack and migraine interval before and after treatment with sumatriptan

Acute myocardial infarction is difficult enough to manage without the complications that commonly occur in the first days and weeks that follow. This article covers the most frequent complications, outlining when they are likely to occur, what the symptoms are, how they are best treated, and what sort of prognosis can be expected. In this way, the author provides a "map" of the days after infarction, showing the pitfalls and sand traps of clinical management.     

Migrainous disorder and its relation to migraine without aura and migraine with aura. A genetic epidemiological study

Migrainous disorder was analysed in a large population-based study of 4000 forty-year-old males and females. All interviews were conducted by one physician and the diagnostic criteria of the International Headache Society were used. Of the 48 people with migrainous disorder, 40 had migrainous disorder without aura and 9 had migrainous disorder with aura. One person had co-occurrence of migrainous disorder with and without aura. The lifetime prevalence of migrainous disorder was 2.5% with a male:female ratio of 1:1.2. The first-degree relatives of probands with migrainous disorder were blindly interviewed. Compared with the general population, first-degree relatives of probands with migrainous disorder without aura had a slightly but less increased risk of migraine without aura than first-degree relatives of probands with migraine without aura. First-degree relatives of probands with migrainous disorder with aura had no increased risk of migraine with aura. We conclude that migrainous disorder without aura in some people is a type of migraine without aura and in other people not. Migrainous disorder with aura may be unrelated to migraine with aura.     

Prophylactic treatment of migraine with gamma-linolenic and alpha-linolenic acids

Polyunsaturated fatty acids (PUFA) were administered to 168 patients over a period of 6 months in an open-label uncontrolled study. In 129 patients available for study, 86% experienced reduction in severity, frequency and duration of migraine attacks, 22% became free of migraine and more than 90% had reduced nausea and vomiting. Self-medication changed to simple analgesics in the majority except in 14% of patients without improvement.     

Familial idiopathic intracranial hypertension with spinal and radicular pain

OBJECTIVE: To describe a mother and her 2 sons affected by idiopathic intracranial hypertension (IIH), associated in the sons with root irritation symptom. Unlike the other 4 families reported previously, obesity was not present in our patients. DESIGN: Case reports. SETTING: Department of pediatrics in a university school of Medicine, Naples, Italy. PATIENTS: A mother (aged 36 years) and her 2 sons (aged 14 and 9 years) developed IIH at different times. Neuroimaging showed an empty sella in the mother, while IIH was associated with spinal and radicular pain in her 2 sons. The mother and the younger son developed permanent visual loss. CONCLUSIONS: Ophthalmologic follow-up in our patients indicates that IIH is a chronic disease. Surgical treatment should be considered an option.     

Retinitis pigmentosa associated with nevus flammeus and unilateral glaucoma

A 53-year-old white man was noted to have bilateral retinitis pigmentosa associated with a left nevus flammeus, a left choroidal hemangioma and unilateral glaucoma. No central nervous system manifestations of Sturge-Weber syndrome were noted. The association of retinitis pigmentosa and the nevus flammeus is probably a chance occurrence.     

Comparison of perirhinal cortex ablation and crossed unilateral lesions of the medial forebrain bundle from the inferior temporal cortex in the rhesus monkey: Effects on learning and retrieval.

Seven monkeys learned new object-reward associations and scene problems and were overtrained on 100 problems of each type. Four monkeys received crossed lesions of the medial forebrain bundle (MFB) and inferior temporal cortex, with the later addition of a fornix section ipsilateral to the MFB lesion. The remaining 3 monkeys received bilateral perirhinal cortex ablation. Disconnection of the MFB from the inferior temporal cortex impaired postoperative new learning, but the retrieval of problems overtrained preoperatively was relatively preserved. Subjects with perirhinal cortex ablation were severely impaired in new learning and at the retrieval of scene problems, but retention of object-reward associations was relatively well preserved. The results support the hypothesis that isolation of the inferior temporal cortex from basal forebrain and midbrain afferents results in dense anterograde amnesia, whereas the role of the perirhinal cortex in learning is dependent on the perceptual difficulty of the task. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Gadolinium and contrast medium MRI of the acoustic nerve in patients with meningeal neuritis and acoustico-facial syndrome

Twelve cases of vestibular neuritis were investigated in gradient echo MRI with gadolinium. Only 3 severe cases associated with an acoustico facial syndrome (2 cases of herpes zoster oticus and one case after influenzae) demonstrated focal enhancement within the internal auditory canal on post contrast T1 weighted images. This enhancement involved at least 2 differents nerves. These 3 severe cases associating sensory neural hearing loss and facial palsy revealed a meningeal reaction after cerebrospinal fluid examination. The enhancement lasted a long time (up to 10 months) in one case of RAMSAY HUNT syndrome associated with a chronic lymphocytic leukemia. The MRI was able to confirm the anatomical reality of the vestibular neuritis and more precisely of the meningoneuritis and gave arguments for the theory of the polyneuropathy of Adour. Enhancement at MRI seems correlated with the severity of the affection (permanent vestibular areflexia in 3 cases and permanent hearing loss in 1 case).     

Relationship between cerebellar appearance and function in children with Dandy-Walker syndrome

We investigated factors relating to mercury sensitization in 156 medical students (mean age 22.7 +/- 2.4, mean +/- S.D., male 113, female 43). Their allergic symptoms, lifestyles and family histories were studied by questionnaire. Patch tests were performed on them with HgCl2 (0.05%aq.), NiSO4 (5%aq.), PPD (2%pet.) and urushiol (0.01%pet.). Anti-dermatophagoides and anti-cryptomeria pollen IgE antibodies in serum were also measured. While the positive rates of urushiol, nickel and PPD were 11.1%, 5.1% and 2.6%, respectively, that of mercury was as high as 12.8%. Each allergen specific antibody positivity and past histories of allergic diseases were not associated with mercury sensitization (by the chi-square test). Mercury sensitized students had significantly more frequently experienced eczema caused by cosmetics, shampoos, soaps and haircreams (by the chi-square test, p < 0.005). They also had significantly more teeth treated with metals compared to the controls (one-tailed t-test, p < 0.05). And their urinary mercury concentrations were significantly higher than those of the controls (one-tailed t-test, p < 0.05). These findings suggest that mercury sensitization is associated with exposure to mercury in the living environment.     

Mammillary body and cerebellar shrinkage in chronic alcoholics with and without amnesia

Mammillary body and cerebellar atrophy have been described as postmorten neuropathologic markers of Korsakoff's syndrome. This study examined whether shrinkage in the mammillary bodies and cerebellum is present consistently in amnesic chronic alcoholics during life and whether the degree of abnormality in these patients differs from that in nonamnesic alcoholic and healthy controls. The severity of shrinkage in the mammillary bodies, cerebellar hemispheres, and cerebellar vermis visualizable on MRI scans was rated on a three-point scale in 33 chronic nonamnesic alcoholics, 9 amnesic alcoholics, and 20 healthy controls. Although both alcoholic groups showed significant mammillary body and cerebellar shrinkage relative to controls, the two patient groups did not differ from each other. Furthermore, four of eight amnesic patients in our sample did not demonstrate clinically significant mammillary body atrophy. These results suggest that alcoholism is associated with mammillary body and cerebellar tissue volume loss but do not provide evidence that these markers distinguish accurately between amnesic and nonamnesic patients. In addition, they suggest that visualizable mammillary body atrophy is not necessary for the development of amnesia in alcoholic patients.     

A correlation of 5-hydroxytriptamine and cerebral vasoreactivity in patients with migraine

Cerebral haemodynamics and the level of serum 5-hydroxytriptamine (5-HT) were analysed in 21 migraine patients (8 with and 13 without aura) during the headache-free period as well as the attack. Cerebral vasoreactivity was evaluated by acetazolamide test and Transcranial Doppler ultrasonography (TCD). TCD findings were within normal ranges in the majority of migraine patients. Patients with migraine without aura had higher blood flow velocities (BFV) than patients with aura. Comparison of BVF and pulsatility index (PI) data patterns in migraine with and in migraine without aura during the attack revealed differences indicating cerebral hypoperfusion. It was noticed that systolic BFV decreased in migraine with aura on the headache side while PI increased. In migraine without aura, both systolic BFV and PI increased. 5-HT findings were heterogeneous. Reduction of cerebral vasoreactivity was observed especially in migraine with aura. Our results suggest different pathogenic mechanisms between migraine with and without aura, supporting the neurogenic inflammation theory.