[Foix-Alajouanine syndrome: case report].

In a 52-year-old woman, spinal arteriovenous malformation (AVM) has been associated with what has been known as Foix-Alajouanine syndrome. The pathophysiology of the AV fistula is probably related to increased venous pressure from the AVM plus thrombotic process. The most common initial symptoms are sensory disturbance, pain and leg weakness. Definitive diagnosis of spinal AVMs requires radiographic demonstration of the vascular anomaly. Nevertheless, in this case, suggestive defects of malformations could not be seen, in contrast to the MRI findings and macroscopical and anatomical-pathological lesion. These findings rise our attention, about the need to keep in mind the clinical suspicion of AVM in cases of back pain and motor deficit, and an early surgical conduct in this situation.

Foix-Alajouanine syndrome: case report

Em mulher de 52 anos, malformação artério-venosa medular (MAV) associava-se com a síndrome de Foix-Alajouanine. A fisiopatologia da fístula artéria-venosa provavelmente está relacionada com a pressão venosa aumentada, a partir da MAV associada com o processo trombótico. Os sintomas iniciais mais comuns são distúrbio sensorial, dor e fraqueza do membro inferior. O diagnóstico definitivo de MAV medular requer evidências radiográficas da anomalia vascular. De qualquer modo, neste caso, defeitos sugestivos de malformações não foram detectados, contrastando com os achados de ressonância magnética e lesões macroscópicas e anátomo-patológicas. Estes resultados chamaram a atenção e excluíram outras causas. A alta suspeita clínica de MAV nos levou a uma conduta cirúrgica precoce em benefício do paciente.

[Creutzfeldt-Jakob disease, Heidenhain variant: case report with MRI (DWI) findings]. / Doença de Creutzfeldt-Jakob forma Heidenhain: relato de caso com achados de ressonância magnética e DWI.

Creutzfeldt-Jakob disease (CJD) is a presenile dementia characterized by rapidly progressive mental deterioration, myoclonic jerking, and other less common neurological signs. Few autoctonous cases have been described in Brazil. A 54-year-old white woman, was admitted in our service with a month history of progressive, bilateral cortical blindness. After admission, she developed right partial motor seizures( right facial, upper and lower limbs), she became progressively aphasic( mixed aphasia). Seizures were controlled with phenytoin, but she developed choreoathetotic movements on her right dimidium, with partial control after introduction of chlorpromazine 25 mg q/d. She could no longer stand up or walk due to severe ataxia. The first EEG (October, 2001) showed left hemisphere severe seizure activity (status epilepticus partialis). She was delivered home with enteral nutrition, phenytoin, chlorpromazine and mepacrine 100 mg qd. The following laboratorial tests were negative or normal: blood series, platelets, ESR, kidney and liver function, copper, ceruloplasmin, VDRL, HIV, HTLV-1, lactate, and cerebral DSA (performed in other service).A spinal tap with normal opening pressure was perform and CSF examination was normal. CSF 14-3-3 protein was positive, CSF specific neuronal enolase 7.5 ng/ml(normal). Genetic study of PRNP gene did not disclosed any known mutation. A MRI (October, 2001) showed areas of hyperintense signal (T2 and FLAIR) without Gd-enhancement on T1, in the left temporal lobe and in both occipital lobes; basal ganglia have a normal appearance. DWI imaging showed bright areas at the same sites. An EEG (March, 2002) disclosed a periodical sharp triphasic waves pattern, suggestive of CJD. A second MRI (April, 2002) showed mild generalized atrophy, no ventricular dilatation, and the hyperintense sites disappeared. She remained clinically stable and under use of chlorpromazine and mepacrine until she died due to pulmonary complications on April, 2003.

Doença de Creutzfeldt-Jakob forma Heidenhain: relato de caso com achados de ressonância magnética e DWI / Creutzfeldt-Jakob disease, Heidenhain variant: case report with MRI (DWI) findings

A doença de Creutzfeldt-Jakob (CJD) é uma forma de demência pré-senil de rápida evolução, geralmente fatal em um ano. Casos autóctones no Brasil têm sido raramente descritos assim como achados de ressonância magnética. Mulher, natural de Ponta Grossa PR, branca , 54 anos , foi admitida no serviço em outubro de 2001 com quadro de amaurose bilateral cortical progressiva desde há 1 mês do internamento. Nunca viajou ao exterior e foi somente submetida a uma cirurgia de redução do estômago, para obesidade. História familial sem relato de casos semelhantes. Logo após o internamento a paciente desenvolveu quadro de disfasia mista, hemiparesia flácida direita, com movimentos coreoatetóticos e crises parciais motoras. Paciente evoluiu com quadro demencial progressivo; atualmente, acamada, torporosa, dependente de alimentação enteral, recebendo mepacrina, fenitoína e clorpromazina , estabilizando o quadro até final de maio de 2002. Exames laboratoriais negativos ou normais. Pesquisa de proteína 14-3-3 no líquor foi positiva; enolase-neurônio-específica no líquor foi normal. Estudo genético do gen PRNP não revelou mutação descrita anteriormente. EEG (23/10/2001) revelou intensa atividade irritativa hemisfério cerebral esquerdo. Estudo de ressonância magnética revelou áreas de hipersinal em T2 e FLAIR em regiões temporal esquerda e bioccipital; gânglios da base normal. Imagens de DWI mostraram hipersinal nas mesmas áreas.Outro EEG (15/03/2002) revelou padrão periódico de ondas trifásicas sugestivos de CJD. A paciente fez uso de mepacrina associado a clorpromazina com aparente estabilização do quadro, até seu óbito por complicações infecciosas pulmonares em abril de 2003.

Surgical approaches to the petrous apex: distances and relations with cranial morphology.

Different pathologies may be located at the petrous apex. The primary surgical approaches used to remove such lesions are the pterional, subtemporal, presigmoid, and retrosigmoid. Each has advantages and disadvantages, and the distance to the petrous apex varies with the approach. Anatomical variations in cranial morphology may interfere with these distances. Dolichocephalic skulls have a longer anteroposterior axis than brachycephalic ones. Three hundred computed tomographic scans and 65 dry human skulls were analyzed to determine if cranial morphology could indicate the shortest distance to the petrous apex. The distance between the external cortical table of the skull and the petrous apex in each surgical approach was measured. The lengths of the anteroposterior axis (L) and the widths of the lateral axis (W) were measured to determine the cranial index (W/L x 100). This distance was longest in skulls with a high cranial index (brachycephalic) independent of the approach used. Statistical analysis showed that the distance to the previous apex was longest in the retrosigmoid approach and shortest in the pterional approach in all kinds of skulls. Brachycephalic skulls lose this ellipsoidal shape and the anterior laterolateral diameter is smaller than the posterior laterolateral diameter. Consequently, the distance from the cortical skull table to the petrous apex is shorter in brachycephalic skulls using all surgical approaches described in this article.

CT scan findings in mild head trauma: a series of 2,000 patients.

The present study describes the cranial computed tomography (CT) scan findings of 2,000 cases of mild head trauma (HT) in Curitiba, Southern Brazil. The mean age of the entire series was 30.8 +/-19 years. The overall male to female ratio was 2:1. The most common causes of head injury were interpersonal aggression (17.9%), falls (17.4%), automobile accidents (16.2%), falls to the ground (13.1%) and pedestrian injuries (13 %). Alcohol intoxication was associated with HT in 158 cases (7.9%). A normal CT scan was seen in 60.75% (1215) and an abnormal CT scan in 39.25% (785) of patients. Out of 785 abnormal CT scan, 518(65.9%) lesions were related to HT. The most common CT scan HT related findings were: soft tissue swelling (8.9 %), skull fractures (4.3 %), intracranial and subgaleal hematomas (3.4% and 2.4 %), brain swelling (2 %) and brain contusion (1.2%). Out of 785 abnormal CT scans, 267 (34.1%) lesions were not related to head trauma. Incidental CT scan findings included brain atrophy (5.9%), one calcification (5.2%) several calcifications (2.4%) (probably neurocysticercosis in most cases), ischemic infarct (1.9%) and leukoaraiosis (1.3%). These findings showed the importance of CT scan examination in mild head injuries. Further studies to identify mild HT patients at higher risk of significant brain injury are warranted in order to optimize its use.

CT scan findings in mild head trauma: a series of 2000 patients

The present study describes the cranial computed tomography (CT) scan findings of 2,000 cases of mild head trauma (HT) in Curitiba, Southern Brazil. The mean age of the entire series was 30.8 ±19 years. The overall male to female ratio was 2:1. The most common causes of head injury were interpersonal aggression (17.9 percent), falls (17.4 percent), automobile accidents (16.2 percent), falls to the ground (13.1 percent) and pedestrian injuries (13 percent). Alcohol intoxication was associated with HT in 158 cases (7.9 percent). A normal CT scan was seen in 60.75 percent (1215) and an abnormal CT scan in 39.25 percent (785) of patients. Out of 785 abnormal CT scan, 518(65.9 percent) lesions were related to HT. The most common CT scan HT related findings were: soft tissue swelling (8.9 percent), skull fractures (4.3 percent), intracranial and subgaleal hematomas (3.4 percent and 2.4 percent), brain swelling (2 percent) and brain contusion (1.2 percent). Out of 785 abnormal CT scans, 267 (34.1 percent) lesions were not related to head trauma. Incidental CT scan findings included brain atrophy (5.9 percent), one calcification (5.2 percent) several calcifications (2.4 percent) (probably neurocysticercosis in most cases), ischemic infarct (1.9 percent) and leukoaraiosis (1.3 percent). These findings showed the importance of CT scan examination in mild head injuries. Further studies to identify mild HT patients at higher risk of significant brain injury are warranted in order to optimize its use