Incarcerated transdiaphragmatic intercostal hernia preceded by Chilaiditi's syndrome.

Transdiaphragmatic hernia most often develops after blunt or penetrating thoracoabdominal trauma. We report on the case of a 73-year-old man who underwent emergency ileocoecal resection for an incarcerated transdiaphragmatic intercostal hernia. The patient's history included both a lumbotomy for right nephrectomy and Chilaiditi's syndrome. The literature regarding both transdiaphragmatic intercostal herniation and Chilaiditi's syndrome is reviewed in relation to the presented case.

Transient myelopathy of the cervical posterior columns in a young man with recently diagnosed diabetes mellitus.

We present the case of a young man with recently manifesting type I diabetes mellitus who progressively developed clinical and radiological signs and symptoms of cervical posterior column myelopathy with eventually spontaneous and complete recovery as concerns both clinical symptomatology and MRI abnormalities.

Pachymeningitis luetica: a case report.

Pachymeningitis luetica is extremely rare in developed countries. We describe a 41-year-old male patient with pachymeningitis luetica, multiple ischaemic infarctions, and severe hydrocephalus. The delay in making the diagnosis contributed to patient's death. Rapid diagnosis is essential on the slightest suspicion of an infection by Treponema pallidum, because timely treatment with antibiotics is effective.

Meningioma presenting as Tolosa-Hunt syndrome.

A 23-year-old woman was admitted with headache, nausea, vomiting and blurred vision on the left side. Neurological examination showed ptosis with a complete internal and external ophthalmoplegia and a red fullness around the left orbita. Computed tomographic scanning of the brain revealed no abnormalities. As she improved on high doses of steroids a diagnosis of Tolosa-Hunt syndrome (THS) seemed to be indicated. However, magnetic resonance imaging (MRI) showed a lesion with intermediate signal intensity in the left cavernous sinus. Craniotomy was performed when symptoms of THS recurred. Histopathological examination revealed a meningioma with a papillary aspect and some mitoses. This case illustrates that: (1) THS is still a diagnosis by exclusion; (2) MRI and histopathological examination are important if there is any doubt about the diagnosis; and (3) also when there is no doubt, improvement after steroid therapy may be a diagnostic pitfall. Therefore, not only MRI but also orbital phlebography and angiography should seriously be considered.

Early onset cerebellar ataxia with retained tendon reflexes: foot deformity in a first grade family member.

Early onset cerebellar ataxia with retained tendon reflexes (EOCA) is a clinical syndrome characterised by progressive cerebellar ataxia with an onset before the age of 25 years and a wide spectrum of associated features. It is distinguished from Friedreich's ataxia (FA) mainly by the preservation of tendon reflexes, a better prognosis, and the absence of GAA expansion in the frataxin gene. Although EOCA is thought to be a hereditary disorder with an autosomal recessive mode of inheritance, genetic heterogeneity might underlie the spectrum of clinical features. In this case report we describe a patient with EOCA accompanied by pes cavus, hammer toes and peripheral neuropathy. The patient's father did not have any ataxia, but had the same foot deformities as his daughter and a slight peripheral neuropathy. The possible relationship between these clinical features is discussed.

Extended extradural spinal arachnoid cyst: an unusual cause of progressive spastic paraparesis.

A 15-year old girl presented with a slowly progressive spastic paraparesis since the age of 12. Creatine kinase was slightly increased. Muscle biopsy carried out during tendon surgery for severe toe-walking showed 'myopathic' changes. Subsequent neurological evaluation and radiological studies revealed a large extradural arachnoid cyst extending from the 11th thoracic vertebra to the first lumbar vertebra. Her condition improved after operation. The 'myopathic' features turned out to be the result of chronic spinal compression. MRI is the method of choice to examine patients with non-hereditary progressive spastic paraparesis. Muscle biopsy and tendon surgery should not be performed, without careful neurological examination.

MRI locates a posterior fossa aneurysm in the fourth ventricle.

We report a rare case of a ruptured aneurysm of the choroidal branch of the left posterior inferior cerebellar artery (PICA) located in the fourth ventricle. Digital subtraction angiography revealed this PICA aneurysm but the exact location remained unknown. The unique location in the fourth ventricle was subsequently shown by magnetic resonance imaging (MRI). The patient died and the final diagnosis was confirmed by autopsy. To our knowledge, this is one of the few reported cases of a PICA aneurysm in the fourth ventricle and the only one which was confirmed by the combination of MRI and autopsy.

Primary cerebellar nocardiosis and alveolar proteinosis.

We report a patient with known asymptomatic pulmonary alveolar proteinosis (PAP) who developed a cerebellar gait disorder and dysarthria caused by an isolated cerebellar nocardial abscess. To our knowledge only 1 patient with PAP and isolated central nervous system nocardia infection has previously been reported. In this early report, diagnosis was established at autopsy. In our patient the clinical and MRI examinations of this cerebellar abscess are described and specific features leading to earlier diagnosis and successful treatment are presented.

Spondylodiscitis in ankylosing spondylitis, inflammation or trauma? A description of six cases.

An uncommon, but well recognised complication of Ankylosing Spondylitis (AS) is spondylodiscitis, a destructive discovertebral lesion also called the Andersson lesion. We describe six cases, with variable clinical presentation and radiological appearance. Two had multiple lesions, in one patient spondylodiscitis was the presenting symptom of AS. None had a history of even a minor trauma. Prognosis was good with conservative treatment including NSAID's, rest, and physiotherapy. In two cases histopathology was studied and suggested sterile inflammation as the main etiologic factor. The literature is reviewed regarding the mechanisms that may contribute to these lesions: mainly inflammatory like increasing enthesopathy or mainly mechanical like pseudoarthrosis about a fracture site. It may be that both mechanisms can result in similar destructive intervertebral disc lesions.

A study of multiple sclerosis patients with magnetic resonance spectroscopic imaging.

Eleven patients with clinically definite MS and three healthy controls were investigated by magnetic resonance spectroscopic imaging. The data sets were analysed for all voxels containing white matter only. We classify these voxels in healthy controls as normal white matter (NWM), and in MS patients as normal-appearing white matter unaffected by MS lesions (NAWM) or white matter affected by MS lesions. The spectra belonging to the voxels were analysed for content of cholines, creatines and N-acetylaspartate (NAA), and compared as a group. It was found that lesions differ from white matter in chemical composition and, moreover, that normal-appearing white matter differs from healthy white matter. Specifically, levels of NAA are lower in patients. There seems to be a linear relation between the composition of white matter and the expanded disability status scale value for the patient. The presence of lactate could not be established, and no unambiguous differences were found between patients with relapsing-remitting and relapsing-progressive disease.

The association of cortical dysplasia and anterior horn arthrogryposis: a case report.

We describe a 21-year-old woman with neurogenic congenital contractures (arthrogryposis) of the lower limbs, normal intelligence, hyper-reflexia and partial epilepsy. MRI revealed bilateral opercular (perisylvian) cortical dysplasia with infolding of cerebral cortex, a focal neuroblast migrational disorder. This type of migrational disorder is known to have a prenatal onset after the 20th fetal week, whereas the anterior horn cell degeneration responsible of neurogenic arthrogryposis originates at 12-14 weeks of gestation. A prenatal viral infection along the neural axis during both these gestational periods or a genetic defect could be responsible for both lesions in this case.

[Spondylodiscitis in Bechterew's disease; inflammation or trauma? Description of 6 patients]. / Spondylodiscitis bij de ziekte van Bechterew; ontsteking of trauma? Een beschrijving van 6 patiënten.

OBJECTIVE: To study the prevalence and nature of sterile spondylodiscitis in patients suffering from ankylosing spondylitis. DESIGN: Descriptive. SETTING: Department of Rheumatology, Medisch Spectrum Twente, Enschede, the Netherlands. METHOD: Of all ankylosing spondylitis patients suffering from sterile spondylodiscitis, the medical histories and the radiological and histological findings were analysed. RESULTS: Among about 400 patients, 6 cases of sterile spondylodiscitis were found; 4 men and 2 women. The mean time lapse between diagnosis of ankylosing spondylitis and onset of spondylodiscitis symptoms was 7 years; in I patient discitis was the first symptom. Discitis changed the nature of the backache: it worsened during exercise and improved on resting. There was no history of trauma. Symptomatic and asyptomatic radiological abnormalities were seen at the same time in 2 patients. Symptoms disappeared 3-36 months after start of conservative treatment. Histological examination was performed in 2 cases and showed inflammatory changes. CONCLUSION: Spondylodiscitis in ankylosing spondylitis has a highly variable presentation and in general a good prognosis. Our findings support an inflammatory rather than a traumatic pathogenesis.

Bacterial arthritis in a district hospital.

Between 1977 and 1988 in the Enschede hospital 72 patients were seen with bacterial arthritis of one or more joints. Staphylococcus aureus was most frequently the causative agent (52%) and the knee was the most frequently infected joint (42%); the mortality rate was 11%. Complete restoration of pre-existent function was seen in 52% of the affected joints. In patients with severe deterioration of joint function after the bacterial infection, the period between the first symptoms and start of treatment (mean 30 days) was significantly longer than in patients with no or moderately deteriorated joint function (mean 10 days). The primary focus was mostly a skin infection, predominantly localized on the lower extremities. Half of all cases of bacterial arthritis occurred in patients with rheumatoid arthritis (RA). We therefore conclude that patients with RA and skin infections, especially if localized on legs or feet, should be treated without delay and that one should not hesitate to prescribe antibiotics. Erythrocyte sedimentation rate (ESR) was less than 20 mm after one hour in 13% and blood leucocyte count less than 10 x 10(9)/liter in 55% of all patients, showing that a normal ESR and/or blood leucocyte count do not exclude bacterial arthritis. In 4 out of 9 patients with infected prosthetic joints the infection resulted in loosening of the joint, before antibiotic treatment was started. In the other 5 patients bacterial arthritis recurred, in one patient resulting in loosening of the joint, only shortly after stopping long-term successful antibiotic treatment (6-24 months). Thus, we feel that lifelong treatment with antibiotics is a reasonable alternative in cases, where the risk of surgery is very high.

Sternocostoclavicular hyperostosis or pustulotic arthroosteitis.

We describe a 68-year-old woman who had suffered pain, swelling, heat, and redness in the region of both clavicles for the last 2 years. Her erythrocyte sedimentation rate was markedly elevated; tests for rheumatoid factor were negative. At surgical exploration, ankylosis of the sternoclavicular joints, especially on the left side, was found. Biopsy revealed chronic nonspecific inflammation with new bone formation, consistent with the diagnosis of sternocostoclavicular hyperostosis or pustulotic arthroosteitis.

Sciatic nerve compression by an aneurysm of the internal iliac artery.

A 73-year-old female with severe sciatica suffered from an aneurysm of the left internal iliac artery. At first minimal caudographic abnormalities suggested intervertebral disc herniation and lumbar root compression but at exploratory surgery the diagnosis had to be rejected. Once the correct diagnosis was established by echography, CT-scanning and angiography surgical treatment of the aneurysm resulted in complete recovery. Because sciatic nerve lesions due to aneurysms in the pelvic region very seldom occur and may be the cause of diagnostic confusion, symptoms and treatment of these aneurysms are discussed.