Jayme Antunes Maciel

Headache during gestation: evaluation of 1101 women.

OBJECTIVE The purpose of this study was to evaluate the presence of headache in women with a previous history or new-onset headache during the current gestation, classify the findings, and describe the clinical characteristics and outcome of the headache. METHODS From January/1998 to June/2002 we prospectively evaluated 1101 pregnant women (12-45 years old), with a history of headache, at two prenatal clinics and an inpatient obstetric public hospital. Women were interviewed using a semi-structured questionnaire during the first, second, and third gestation trimesters and immediately after delivery. All interviews were conducted by one of the authors, using the International Headache Society Classification (IHSC-2004). RESULTS In 1029 women there was a history of headache prior to the current pregnancy, 36 (3.4%) women first experienced headache during this pregnancy and 40 patients experienced new types of headache. In these 76 patients with new onset headache during pregnancy, 40 had secondary headache (52.6%), 31 had primary headache (40.8%), and 5 had headache not classified elsewhere (6.6%). According to IHSC- 2004 criteria, we found migraine in 848/1029 women (82.4%), with pregestational headache. CONCLUSIONS Most of the pregnant women presented with headache, mainly in migraine, prior to pregnancy, and most of the headaches improved or disappeared during the second and third gestation trimester. In a relatively small number of pregnant women, a new type of headache started during the gestation.

Gabapentin-responsive idiopathic stabbing headache.

Idiopathic stabbing headache (ISH) is defined as the occurrence of short-lasting, painful jabs, restricted to the ophthalmic division of the trigeminal nerve. It is closely related to other forms of headache (such as migraine and tension-type headache) and has been reported among all age groups, including children and adolescents. As pathogenic mechanisms of the disease remain unclear, management decisions are empirical and limited to few options. Classically, indomethacin has been considered the first option, but therapeutic failure occurs in up to 35% of cases. In this setting, we report four patients with young-onset indomethacin-resistant ISH which had good responses to gabapentin and discuss the use of this drug in the presenting situation.

Headaches during pregnancy in women with a prior history of menstrual headaches

OBJECTIVE To evaluate the presence of menstrual headaches prior to pregnancy according to the International Headache Society (IHS) classification criteria, 2004, and also study the outcome (frequency and intensity) of these pre-existing headaches during the gestational trimesters. METHOD This study involved 1,101 pregnant women (12 to 45 years old). A semi-structured questionnaire was used to interview the women during the first, second and third gestational trimesters as well as after delivery. All the interviews were conducted by one of the researchers by applying the IHS Classification (IHSC-2004). RESULTS A 1,029 women out of the 1,101 women interviewed presented headaches prior to gestation, which made it possible to study headaches in 993 women during the gestational trimesters. Menstrually related headaches were presented by 360 of the 993 women. Migraine was reported by 332/360 women (92.22%) with menstrual headaches and 516/633 women (81.51%) without menstrual headaches, respectively, prior to gestation. The majority of the women with menstrual migraine presented a headache improvement or disappearance during gestation (62.22% during the first trimester; 74.17% during the second trimester; 77.78% during the third trimester). CONCLUSION Most of the pregnant women with menstrual or non-menstrual headaches prior to gestation presented migraine, which either improved or disappeared during pregnancy. Women who suffered from non-menstrual headaches improved during pregnancy but not as much as women with menstrual headaches.

Orbital apex syndrome due to aspergillosis: case report

We report the case of a 73-year-old female who presented facial numbness and pain in the first division of the trigeminal nerve, ptosis, diplopia and visual loss on the right side for the previous four months. The neurological, radiological and histological examination demonstrated a rare case of invasive fungal aspergillosis of the central nervous system, causing orbital apex syndrome, later transformed in temporal brain abscess. She died ten months later due to respiratory and renal failure in spite of specific antimycotic therapy.

Molecular characteristics of Machado-Joseph disease mutation in 25 newly described Brazilian families

Machado-Joseph disease (MJD) is a form of autosomal dominant spinocerebellar ataxia first described in North-American patients originating from the Portuguese islands of the Azores. Clinically this disorder is characterized by late onset progressive ataxia with associated features, such as: ophthalmoplegia, pyramidal and extrapyramidal signs and distal muscular atrophies. The causative mutation is an expansion of a CAG repeat in the coding region of the MJD1 gene. We have identified 25 unrelated families segregating the MJD mutation during a large collaborative study of spinocerebellar ataxias in Brazil. In the present study a total of 62 family members were genotyped for the CAG repeat in the MJD1 gene, as well as 63 non-MJD individuals (126 normal chromosomes), used as normal controls. We observed a wide gap between the size range of the normal and expanded CAG repeats: the normal allele had from 12 to 33 CAGs (mean = 23 CAGs), whereas the expanded alleles ranged from 66 to 78 CAGs (mean = 71.5 CAGs). There were no differences in CAG tract length according to gender of affected individuals or transmitting parent. We observed a significant negative correlation between age at onset of the disease and length of the CAG tract in the expended allele (r = -0.6, P = 0.00006); however, the size of the expanded CAG repeat could explain only about 40% of the variability in age at onset (r2 = 0.4). There was instability of the expanded CAG tract during transmission from parent to offspring, both expansions and contractions were observed; however, there was an overall tendency for expansion, with a mean increase of +2.4 CAGs. The tendency for expansion appeared to the greater in paternal (mean increase of +3.5 CAGs) than in maternal transmissions (mean increase of +1.3 CAGs). Anticipation was observed in all transmissions in which ages at onset for parent and offspring were known; however, anticipation was not always associated with an increase in the expanded CAG repeat length. Our results indicate that the molecular diagnosis of MJD can be confirmed or excluded in all suspected individuals, since alleles of intermediary size were not observed.

Pseudocystic form of neurocryptococcosis in pregnancy: case report

We report a case of neurocryptococcosis which is unique in the literature because the patient had a pseudocystic form of the disease during pregnancy and without any evidence of AIDS. The clinical picture was that of intracranial hypertension and the epidemiological background was highly suggestive of cysticercosis. CT showed multiple round hypodense lesions in the basal ganglia and cerebellum, without contrast enhancement. Since a scolex was not visible, the diagnosis of neurocysticercosis was considered probable. CSF examination was not performed in view of its high risk. The patient had progressive downhill course. Autopsy disclosed multiple gelatinous pseudocysts in the cerebral and cerebellar gray matter, containing abundant Cryptococcus neoformans. Meningeal involvement was minimal. The child was delivered by caesarean section and was free of infection, but died later of hyaline membrane disease. The neuroimaging appearances of this rare instance of the pseudocystic form of neurocryptococcosis mimicked closely neurocysticercosis and only postmortem examination allowed correct diagnosis. The pseudocystic form has so far only been reported in AIDS.

Vasopressin serum levels in patients with severe brain lesions and in brain-dead patients

INTRODUCTION Patients with severe brain lesions (SBL) and brain-dead patients (BD) frequently present with vasopressin (AVP) secretion disorders. OBJECTIVE To evaluate AVP serum levels in SBL and BD patients. DESIGN Prospective, open label, observational trial. SETTING A general teaching hospital. METHOD Three groups of adult subjects (age> 18y) of both sexes were included in this study: control group: 29 healthy volunteers; SBL group: 17 patients with Glasgow Coma Scale (GCS)< 8; and BD group: 11 brain-dead patients. Samples of venous blood were collected in the morning at rest from healthy volunteers and at 8 hourly intervals over a period of 24h from SBL and BD patients for AVP determinations. Concomitantly, some clinical and laboratorial variables were also recorded. RESULTS AVP serum levels (pg/ml) were [mean (SD); median]: control [2.2(1.1); 2.0]; SBL [5.7(6.3); 2.9]; and BD [2.6(1.0); 2.8]. AVP serum levels varied greatly in SBL patients, but without statistically significant difference in relation to the other groups (p=0.06). Hypotension (p=0.02), hypernatremia (p=0.0001), serum hyperosmolarity (p=0.0001) and urinary hypoosmolarity (p=0.003) were outstanding in BD patients when compared with SBL. CONCLUSIONS The AVP serum levels did not demonstrate significant statistical difference between the groups, only showing a greater variability in SBL patients (manifested as serum spike levels). Hypernatremia and hyperosmolarity were present in BD patients, indicating a failure of the hypothalamic-pituitary system in AVP production and release.

PILONIDAL CYST ON THE VAULT: CASE REPORT

Pilonidal cysts and sinuses are described as dermoid cysts which contain follicles of hairs and sebaceous glands. They clinically present as a classic case of inflammation which comes with pain, local infection and redness. The origin of pilonidal disease remains controverse. There are many hypothesis as lack of hygiene on the affected area and a penetration and growth of a hair in the subcutaneous tissue caused by constant friction or direct trauma on the damaged area. The option for clinical treatment is very frequent. However, taking into consideration the incidence and the possibility of recidive, surgical treatment is presently recommended. Complications include cellulitis and abscess formation. Pilonidal cysts are mostly found on the sacral region. In the literature is found description of pilonidal cysts on the penis, interdigital region on the hands as well as on the cervical region. We present a case of pilonidal cyst located on the vault biparietal region, without malignant degeneration.

Recommendations for the treatment of migraine attacks - a Brazilian consensus.

In this article, a group of experts in headache management of the Brazilian Headache Society developed through a consensus strategic measurements to treat a migraine attack in both the child and the adult. Particular emphasis was laid on the treatment of migraine in women, including at pregnancy, lactation and perimenstrual period.

Intelligence functions disorders in patients with complex partial epilepsy

OBJECTIVES To compare the performance of patients with complex partial epilepsy with the normal controls in the subtests of an instrument used to assess intelligence function. METHOD Fifty epileptic patients, whose ages ranged from 19 to 49 years and 20 normal controls without any neuropsychiatric disorders. The Wechsler-Bellevue adult intelligence test was applied in groups, epileptic patients and control subjects. This test is composed of several subtests that assess specific cognitive functions. A statistical analysis was performed using non-parametric tests. RESULTS All the Wechsler-Bellevue subtests revealed that the intelligence functions of the patients were significantly inferior to that of the controls (p<0.05). This performance was supported by the patients complaints in relation to their cognitive performance. CONCLUSION Patients with complex partial epilepsy presented poorer results in the intelligence test when compared with individuals without neuropsychiatric disorders.