Brazilian experience of the influence of endovascular treatment on headache in patients with ruptured intracranial aneurysms.

BACKGROUND: The characteristics of primary headaches in patients with ruptured brain aneurysm embolization have not been well understood to date. AIMS OF THE STUDY: This study was conducted to evaluate the influence of endovascular treatment (EVT) of ruptured intracranial aneurysm (RIA) and the pattern of previous primary headache. METHODS: We evaluated the pattern of headache in patients who suffered a RIA and EVT within one year before the rupture, and prospectively evaluated the characteristics of headache for up to 12 months after EVT. Sixty patients were evaluated and a questionnaire about headache was applied at the time of admission. These patients were contacted 3, 6 and 12 months after treatment to complete the study by filling out follow-up questionnaire on the headache. RESULTS: Thirty-seven patients had headache before the rupture, 16 with tension-type headache (TTH), 11 with migraine without aura, nine with migraine with aura with and one with non-specific characteristics. There was a significant reduction in the frequency of pain for up to 12 months for patients with TTH (P < 0.001) and migraine without aura (P = 0.012), but there was a reduction in pain intensity over the same period in TTH (P = 0.002), migraine with aura (P = 0.004) and migraine without aura (P < 0.001). CONCLUSION: There was a reduction of the primary headache after endovascular treatment of ruptured brain aneurysm.

Hamartoma fibrolipomatoso de nervo mediano: relato de caso / Fibrolipomatous hamartoma of the median nerve: case report

Fibrolipomatous hamartoma is a rare benign neoplasm that in some cases is associated with macrodactylia. We describe a 31-year-old man who had a tissue enlargement in the wrist, second and third fingers of the left hand since infancy. At 23-years-old he began with continuous, progressive and high intensity pain that occurred more frequently at night, localized in the left hand. It was associated with paraesthesias and hypostesias predominantly at the fingers described above. Investigation with X-ray, ultrasonography, electrodiagnosis, magnetic resonance image of the left wrist and hand showed carpal tunnel syndrome with macrodactylia by fibrolipomatous hamartoma of the median nerve. The patient did not a have good response to clinical therapy, so he was submitted to a surgical decompression of the left carpal tunnel, and after three months of follow up is asymptomatic.

O hamartoma fibrolipomatoso é neoplasia benigna rara que em alguns casos esta associada com macrodactilia. Descrevemos o caso de homem de 31 anos que apresentava desde o nascimento aumento de volume em região de punho, segundo e terceiro quirodáctilos da mão esquerda. Aos 23 anos iniciou dor contínua, de forte intensidade, predominante no período noturno e de evolução progressiva em mão esquerda. Associada à dor havia hipoestesia e parestesias de predomínio nos segundo e terceiro quirodáctilos esquerdos. A investigação complementar com radiografia, ultrassonografia, estudo eletrofisiológico e ressonância magnética de mão e punho esquerdos confirmaram a suspeita de síndrome do túnel do carpo secundária a macrodactilia com hamartoma fibrolipomatoso do nervo mediano. O paciente foi submetido à descompressão cirúrgica do túnel do carpo esquerdo devido a ausência de resposta ao tratamento clínico e evoluiu com melhora dos sintomas em avaliação após três meses do procedimento.

Estudo da condução nervosa motora na doença de McArdle: relato de caso / Motor nerve conduction study in McArdle disease: case report

McArdle disease (glycogenosis type V) is a metabolic myopathy with symptoms of exercise intolerance caused by deficiency of the enzyme myophosphorylase. In these patients, the motor nerve conduction studies after a short period of maximal voluntary muscle contraction or repetitive stimulation reveals characteristic findings of the disease. A 37-year-old man presented symptoms of exercise intolerance, muscular fatigue and cramps in the beginning of the physical activity with [quot ]second wind[quot ] phenomenon. The motor nerve conduction studies after a voluntary contraction of 30 and 90 seconds presented decrease in the amplitude of the compound muscle action potential in median, ulnar and deep peroneal nerves; and decrement after 200 stimulation at the 40 Hz in deep peroneal nerve. The electromyography presented myopathic pattern and during the ischemic exercise electric silence was not proven. The characteristic of electrophysiological studies are discussed with emphasis at the importance of the motor nerve conduction studies in the patients with suspicion of metabolic myopathy.

A doença de McArdle (glicogenose tipo V) é miopatia metabólica com sintomas de intolerância ao exercício, causados pela deficiência da enzima miofosforilase. Nesses pacientes, o estudo da condução nervosa motora após período de esforço muscular máximo ou ao estímulo repetitivo pode revelar achados característicos da doença. Descrevemos o caso de um homem de 37 anos com sintomas de intolerância aos exercícios, fadiga muscular e cãibras no início da atividade física com a presença do fenômeno de "second wind". O estudo da condução nervosa motora apresentava redução na amplitude do potencial de ação muscular composto após esforço de 30 e 90 segundos em nervos mediano, ulnar e fibular profundo e decremento após 200 estímulos a 40 Hz em nervo fibular profundo. A eletromiografia de agulha apresentava padrão miopático e durante o exercício isquêmico não se evidenciou silêncio elétrico. Discutimos as características eletrofisiológicas enfatizando a importância do estudo da condução nervosa motora e teste de estimulação repetitiva nos pacientes com suspeita de miopatia metabólica.

VARIABILITY IN THE PRODUCTION OF Mus domesticus domesticus EMBRYOS. / VARIABILIDADE NA PRODUÇÃO DE EMBRIÕES DE Mus domesticus domesticus.

The aim of this experiment was to verify the variability of the Mus domesticus domesticus (CF1 strain) to produce viable embryos. We collected 4196 structures from 74 females individually mated with five (5) different males. The zygotes of each female donor were cultured in vitro in KSOM medium incubated at 37ºC in a 5% CO2 air humidified atmosphere for 120 h. Females were distributed in five groups (G1- G5) according to the number of collected structures and the number of superovulatory treatments that they were submitted. Analysis of data showed a variability in the embryo production capacity among the females and in response to the first superovulatory treatment (p< 0.05). The males also played a role in the results (p< 0.05). Thereby, male number five (5) presented the greatest potential for embryo production. The embryonic cleavage and the development rates to the blastocyst and hatched blastocyst stage were determined. Results demonstrated variability in the number of produced embryos and their development competence to reach the hatched blastocyst stage taking into account the female donor and the male. KEY-WORDS: Variability. Zygotes. Culture. Cleavage. Preimplantation.

O objetivo do experimento foi verificar a variabilidade na produção de embriões de Mus domesticus domesticus linhagem CF1, determinando as taxas de clivagem e de desenvolvimento embrionário até o estádio de blastocisto e blastocisto eclodido. Foram obtidas 4196 estruturas de 74 fêmeas, acasaladas individualmente com 5 diferentes machos da mesma linhagem. Os zigotos de cada fêmea doadora foram cultivados in vitro em meio KSOM, em atmosfera gasosa úmida com 5 % de CO2 à temperatura de 37ºC durante 120 horas. As fêmeas foram distribuídas em cinco grupos (G1 a G5) de acordo com o número de estruturas coletadas e conforme o número de tratamentos superovulatórios a que foram submetidas. A análise dos dados revelou a existência de variabilidade na produção de embriões e na eficiência (P < 0,05) das fêmeas em resposta ao primeiro tratamento superovulatório. Os machos também influenciaram nos resultados (p < 0,05), destacandose o de número cinco (5) como o de maior potencial na produção de embriões. Os resultados evidenciaram uma variabilidade no número e na capacidade de desenvolvimento dos mbriões Mus domesticus domesticus linhagem CF1, considerandose a fêmea doadora, o macho empregado e a reutilização das fêmeas que fracassaram no primeiro acasalamento.PALAVRAS-CHAVE: Variabilidade. Zigotos. Cultivo. Clivagem. Pré-implantação.

Generalized dystonia and striatal calcifications with lipoid proteinosis.

Lipoid proteinosis (LP) is an autosomal recessive disease that typically presents with papular, verrucous, poxlike, or acneiform scars and lesions and hoarseness. LP was recently mapped to the 1q21 locus and shown to result from mutations in the extracellular matrix protein 1 gene (ECM1). Epilepsy, mental retardation, and hippocampal calcifications can occur. The authors describe a patient with generalized dystonia caused by striatal calcifications.

Evaluation of headache intensity in migrainous patients with visual handicap through the tactile analogical scale (TAS).

The tactile analogue scale (TAS) was elaborated to be used in blind subjects or those who can not use the vision during their crises. The objective of this study was to characterize, from TAS, the architecture of migraine attacks in subjects with visual disability. For that, 11 migrainous with visual disturb (MVD) subjects were studied and 22 migrainous subjects with no visual disability as a control group. All patients fulfilled the criteria for migraine and the patients of the group studied showed visual acuteness less than 20/200. To evaluate the results, the patients of the group MVD were subdivide within two groups, according to their visual acuteness: subgroup A subjects with subnormal vision and subgroup B amaurotic ones. In subgroup A measurement 46 attacks with average of the migraine attacks of the 56.50 mm, in the subgroup B 45 attacks with average of the 59.58 mm and in the control group 92 attacks with average of the 49.88 mm. When subgroup B and control group were compared there was a significant statistic difference (p=0.022). Through these outcomes we can observe that the migrainous subjects with no visual afference show a higher pain intensity during the migraine crises comparing to those subjects with no visual handicap. The study suggests that, as in other forms of sensibility, the total visual loss can also interfere in the nociceptive control of the pain during the migraine attacks.

[Anesthetic blockade of the greater occipital nerve in migraine prophylaxis]. / Bloqueio anestésico do nervo occipital maior na profilaxia da migrânea.

Migraine comprises a great many encephalic structures in its pathophysiology with the trigeminal nerve (TN) type being one of the main ones. For the purpose of determining a possible influence of the greater occipital nerve (GON) on migraine behavior, 37 patients who showed this pathology were studied. Using a double blind "cross over" group and submitting those patients to a GON infiltration with bupivacaina 0.5% (BP) and physiological serum 0.9% (PS), the clinical effects were evaluated: subjectively, through a pain analytical visual scale; objectively, by determining the threshold of pain perception (algometry). The comparison between the two groups (BP-PS) and (PS-BP) has shown that the number and duration of the attacks did not show significant statistical differences during the study. The intensity of the attacks was lower in group (BP-PS) only after the second infiltration (p=0.020), in the other moments no differences have been observed between the groups. The conclusion is that the anesthetic blockage with BP on the GON does not change the number of crises and their duration, but it does provokes an intensity reduction after 60 days from the infiltration. The results shown here suggest that GON participates in the cranial nociceptive modulation during crises of migraine without aura.

Influence of intense light stimulation on trigeminal and cervical pain perception thresholds.

Thirty-three migraineurs and 23 healthy controls were submitted to pressure algometry before and after light-induced discomfort was elicited by progressive light stimulation in a monoblind fashion. Pressure algometries were performed on the emergence of the supraorbital, infraorbital, mental and greater occipital nerves, and over the temporal muscles, always throughout the same sequence and from right to left. Measurements were carried out before and immediately after light stimulation and after 10 min of the second algometry. The final result for each site measured at each time-point was the mean of the three measurements. Light stimulation was carried out progressively until light-induced discomfort was reported, to a maximum of 20,000 lux. A heat-blocking glass protected patients' eyes. Migraineurs presented significant and persistent drops in pain perception thresholds after light stimulation, at all sites tested (P = 0.002 to < 0.0001). These drops were not seen in controls, in whom, conversely, a less significant increase was seen on right infraorbital and left temporal muscle sites. Our results indicate that in migraineurs, light may have a relevant role in trigeminal and cervical pain perception thresholds.

[Pseudomigraine with CSF pleocytosis: intermittent measurement of the intracranial pressure. Case report]. / Pseudomigrânea com pleocitose liquórica: monitorização intermitente da pressão intracraniana. Relato de caso.

Pseudomigraine with pleocytosis is a self-limited and rather benign disorder, characterized by recurrent bouts of migrainous headaches, associated to focal neurological symptoms and to cerebrospinal fluid abnormalities. We have submitted an individual with pseudomigraine to three lumbar punctures. The first and the third lumbar punctures, carried out during symptomatic periods, revealed a cerebrospinal fluid with aseptic lymphomonocytic pleocytosis, and an opening pressure of 400 and 440 mmH2O, respectively. The cerebrospinal fluid pressure measured during an asymptomatic period was normal (190 mmH20). Although the underlying mechanisms of this disorder remain elusive, there is some evidence that suggests an involvement of autoimmune mechanisms leading to a reduction of the cerebral blood flow, similar to that seen in the cortical spreading depression. In this report, we raise the possibility of a contributory role to be played by the elevated cerebrospinal fluid pressure on the pathophysiology of this disorder.

[Prevalence and semiologic aspects of the idiopathic stabbing headache in a migraine population]. / Prevalência e características da cefaléia idiopática em punhaladas em uma população de migranosos.

Idiopathic stabbing headache is a quite unknown disorder. Its main features consist of brief stabbing pains, lasting few seconds. In most cases, idiopathic stabbing headache is underdiagnosed. We have followed up migraine patients during a period of 12 months, aiming to determine the prevalence and main features of idiopathic stabbing headache while occurring apart from migraine attacks. Two hundred and thirty-three of the 280 patients initially surveyed were included in the analysis of the results. Ninety-four patients presented idiopathic stabbing headaches (40.4%), being 72 of them females (76.5%). For migraine with idiopathic stabbing headaches, mean age, age of beginning of migraine and years with migraine were 33, 22.5 and 10.6 years, respectively. Mean duration of the idiopathic stabbing headaches was reported to be 1.42 seconds [ 1 second by 68 patients (72.4%), 2 seconds by 17 (18.1%), 3 seconds by 6 (6.3%), 4 seconds by 1 (1.05%) and 5 seconds by 2 (2.15%)]. Pain paroxysms were reported to be unilateral by 86 (91.4%) and bilateral by 8 (8.6%) of the cases. They were reported to be temporal by 56 patients (60%), occipital by 15 (15.6%), frontal by 8 (8.5%), temporo-occipital by 7 (7.4%), parietal by 5 (5.3%), fronto-temporal by 1 (1.06%), cervical by 1 (1.06%) and ocular by 1 patient (1.06%). The study confirms a high prevalence of idiopathic stabbing headaches in migraineurs. Its main clinical features could be well determined during the interval between migraine attacks.

Referred pain after painful stimulation of the greater occipital nerve in humans: evidence of convergence of cervical afferences on trigeminal nuclei.

Cranial sensory innervation is supplied mainly by the trigeminal nerves and by the first cervical nerves. Excitatory and inhibitory interactions among those nerve roots may occur in a mechanism called nociceptive convergence, leading to loss of somato-sensory spatial specificity. Three volunteers in an experimental trial had sterile water injected over their greater occipital nerve on one side of the neck. Pain intensity was evaluated 10, 30 and 120 s after the injection. Two of the patients reported intense pain. Trigeminal autonomic features, suggestive of parasympathetic activation, were seen associated with trigeminally distributed pain. These data add to and reinforce previous evidence of convergence of cervical afferents on the trigeminal sensory circuit.

[Using algometry of pressure measuring the threshold of trigeminal pain perception in normal volunteers: a new protocol of studies]. / Utilização da algometria de pressão na determinação dos limiares de percepção dolorosa trigeminal em voluntários sadios: um novo protocolo de estudos.

Algometry of pressure is a technique that measures the physiology of the nociceptive system. Acting directly on the responsive peripheral nociceptors to pressure stimuli, this technique allows the study on nociceptive integrity in normal subjects or having different algic syndromes. Utilizing 29 asymptomatic volunteers, the threshold of the painful perception was studied, measuring them in a direct way over the emergence of the supra-orbital, infra-orbital and mental nerves. The following algometric average were recorded: right mental nerve 46.2 Kg/cm2 and left 48.6 Kg/cm2; right supra-orbital nerve 47.7 Kg/cm2 and left 45.2 Kg/cm2; right infra-orbital nerve 53.9 Kg/cm2 and left 55.4 Kg/cm2. After reviewing the principles of the algometry utilization, we have validated this protocol, showing the average values obtained by measuring the trigeminal system, afterwards comparing them with an inervated region by cervical branches (major occipital nerve) and the temporal muscle.

Prevalence and clinical features of migraine in a population of visually impaired subjects in Curitiba, Brazil.

To investigate the relevance of lacking or diminished visual input on the expression of migraine, we evaluated its prevalence and clinical features in a population of visually impaired subjects. Between September 1999 and April 2000, 203 visually impaired subjects with a headache inventory were surveyed. Those with headache were assessed according to IHS criteria for the presence of migraine. Migraineurs had their symptoms further detailed through an interview and a headache diary. Of the 104 subjects reporting headaches during the last 6 months, 29 had migraine (14.2%). The prevalence of migraine was not influenced by whether the visual impairment was complete or partial. Mean frequency of migraine attacks was 2.7/month. Most subjects (96%) reported severe and/or moderate attacks. Nausea, vomiting, aggravation by activity and phonophobia were reported by 62%, 37.9%, 86.2% and 96.6% of the subjects, respectively. Visual impairment does not seem to influence prevalence of migraine or its clinical features.

[Lipoma of the corpus callosum associated with the hypertrophy of the corpus callosum: case report]. / Lipoma do corpo caloso associado a hipertrofia do corpo caloso: relato de caso.

Despite the lipomas are the tumors that more commonly occur in the corpus callosum (CC), its incidence in the population is not common. We report on a 5-year old boy, with history of retardation in the psychomotor development and disturbs in the gait, secondary to generalized hypotony. Magnetic resonance imaging showed a curvilinear lipoma of the CC related to its hypertrophy. In the literature patients with CC lipoma usually have agenesis or hypotrophy of the CC, but in the reported case we have seen, by the first time, a hypertrophy of the CC. We made embryologic, genetic, clinical, radiographic and therapeutic considerations about the patients that have CC lipoma comparing to findings in the case we report.

[Change in the biological pattern of migraine with aura after the use of tetrabenazine: case report]. / Mudança no padrão biológico da migrânea com aura após a utilização da tetrabenazina: relato de caso.

The influence of the dopaminergic system on the pathophysiology of migraine has been suggested as a result of recent genetic discoveries. A possible hyper-reactivity of the dopaminergic receptors DRD2 reinforced the evidence regarding this. We describe a 31 years-old male patient affected by a generalized dystonia secondary to perinatal hypoxia. At age 16, the patient started having headache crises that met the criteria for migraine with aura. After three years of treatment for dystonia with tetrabenazine, a clear reduction in the frequency, intensity and duration of the crises was perceived. During two periods longer than two months, the interruption of the treatment with tetrabenazine brought about an aggravation of the migraine crises. We present this case as being the first description in the literature showing the beneficial effects of tetrabenazine, a blocker of dopaminergic receptors, on the behavior of migraine with aura.

[Can the biologic pattern of cervicogenic headache change after overuse or withdrawal of ergotamine derivatives?]. / O padrão biológico da cefaléia cervicogênica pode ser alterado pelo uso excessivo de ergotamínicos ou pela sua retirada?

The transformation of a primary headache into a chronic daily headache (CDH) may or may not be related to the overuse of pain-killers, as their influence on the pathophysiological mechanisms remain inconclusive. We describe three patients (female, aged 65 and 39 years, and male, 46) affected by cervicogenic headache (CH) and CDH linked to the overuse of pain-killers (ergotamine derivatives) that were submitted to the infiltration of the greater occipital nerve (GON). At the end of three days of treatment, a total improvement of the pain symptoms was recorded, which allowed for the withdrawal of the ergotamine derivatives. The CH cannot be ranked with the CDHs, since it presents an organic etiology; however, if the pain is daily and the diagnosis is belated, the indiscriminate and excessive use pain-killers may occur. In the cases described, the overuse of pain-killers did not affect the natural evolution of this headache after treatment with the infiltration of the GON, as all the patients who underwent infiltration showed a total improvement of their painful symptoms, without headache resulting from the withdrawal of pain-killers, nor did they show any pharmacological dependence. This is an evidence that the CH presents and organic etiology, not being influenced in its pathophysiology by the overuse of ergotamine derivatives.

Prolonged migraine aura without headache arrested by sumatriptan. A case report with further considerations.

The case of a 42-year-old woman with prolonged migraine visual aura without headache, whose long-lasting episodes of visual aura were successfully controlled by oral sumatriptan, is reported. Effectiveness of sumatriptan was unequivocal, since, after taking sumatriptan, duration of aura would drop from 1.5 h to approximately 20 min. This case suggests that sumatriptan may cross the blood-brain barrier and block spreading depression.

[Greater occipital neuralgia associated with occipital osteolytic lesion. Case report]. / Nevralgia do occipital maior associada a lesão osteolítica occipital. Relato de caso.

The anatomic distribution of the greater occipital nerve during its path permits a close relationship with muscular structures, tendons, vessels and bones. The rupture of this relationship can origin its irritation and headache. We describe an uncommon association between an osteolytic lesion on occipital bone and greater occipital nerve. The patient, female 50, has been presenting headache for two years on the right occipital region spreading to the hemicranic and ipsilateral supraorbital region. The symptoms started spontaneously or by pressure on the trapezius tendon. The pain lasted about 30 minutes, compressive, mild intensity, with no autonomic symptoms and no improvement after the infiltration in the greater occipital nerve. The total improvement of the symptoms after releasing the nerve has allowed us to associate this lesion to the presence of algic symptoms.