Bilateral stroke following pituitary apoplexy.

Pituitary apoplexy followed by cerebral infarction is rare. We report a 59-year-old male with a known pituitary macroadenoma who was admitted to our emergency department for treatment of an acute myocardial infarction. He underwent coronary angioplasty and was subsequently treated with aspirin, clopidogrel and full-dose enoxaparin. He developed pituitary apoplexy with bilateral compression of both internal carotid arteries, and infarction of both anterior and middle hemispheres; consequently, he died. This patient illustrates the difficulties of administering aggressive anticoagulative and antiplatelet therapy to patients who have a known pituitary adenoma.

Early mitoxantrone-induced cardiotoxicity in secondary progressive multiple sclerosis.

Mitoxantrone is an anthracenedione antineoplastic agent approved as an escalating immunotherapy for multiple sclerosis. Owing to structural similarity with other anthracyclines, cardiotoxicity is a severe side effect of mitoxantrone. The risk of mitoxantrone-induced cardiotoxicity in patients with multiple sclerosis increases with cumulative doses >100 mg/m(2) body surface area (BSA). However, the effect of mitoxantrone on cardiac function in the early phase of treatment with cumulative doses <100 mg/m(2) BSA is unclear. The present report concerns four patients with a temporary and considerable decrease in left ventricular ejection fraction (LVEF) and with additional echocardiographic findings of diastolic dysfunction after only one or two doses of mitoxantrone. The risk of cardiotoxicity at low doses of mitoxantrone is highlighted.

[Relapsing reversible posterior leukoencephalopathy after chemotherapy with cisplatin and 5-fluorouracil]. / Rezidivierendes reversibles posteriores Leukenzephalopathiesyndrom unter Chemotherapie mit Cisplatin und 5-Fluorouracil.

We report a 37-year-old patient with gastric cancer who suffered two distinct episodes of generalized tonic-clonic seizures during ongoing chemotherapy with cisplatin and 5-fluorouracil. Cranial MRI revealed reversible posterior leukoencephalopathy (RPL) that remitted completely without any specific therapy after discontinuation of the chemotherapeutic drugs. Interestingly, RPL occurred repeatedly in our patient following the single chemotherapy cycles, which has not been described so far. This syndrome is clinically relevant for differential diagnosis of bilateral ischemia of the posterior cerebral arteries. An overview of the literature and necessary diagnostic procedures is given.

A case of recurrent Miller Fisher syndrome mimicking botulism.

Miller Fisher syndrome (MFS) is a rare and usually monophasic polyradiculoneuropathy characterised by ophthalmoplegia, decreased or absent tendon reflexes, and ataxia. The objective of this study was to report a case of recurrent MFS with a clinical presentation virtually indistinguishable from botulism. The patient was a young man with two episodes of increasing external ophthalmoplegia, ptosis, and ataxia with a long asymptomatic interval in between. The second episode occurred after consumption of rotten fish and was accompanied by gastrointestinal symptoms and an anticholinergic syndrome. Very rarely, MFS can present with a recurrent course. The importance of this case of recurrent MFS lies not only in its long asymptomatic period and identical clinical presentation, but also in its instructiveness regarding the differential diagnosis of MFS, particularly life-threatening botulism.

Cardiogenic embolism as the main cause of ischemic stroke in a city hospital: an interdisciplinary study.

BACKGROUND: It is essential to understand the pathogenesis of ischemic stroke to ensure rational acute therapy and secondary prevention. We wanted to know the distribution of pathogenesis in patients of a city hospital and the differences in risk factors, neurologic deficits, disability, and delay in clinical admittance. PATIENTS AND METHODS: During a period of one year, 222 patients (mean age 76.6 years; 59% women) with complete acute ischemic stroke were admitted and underwent complete clinical and diagnostic procedures: CCT/MRI; Doppler- and color-coded duplex and transcranial sonography; echocardiography; use of the NINCDS stroke scale and the Oxford disability scale; study of risk factors, and exploration of delay in admittance. RESULTS: The following percentages of etiologies were evident: 31% cardiogenic embolism (60% with atrial fibrillation), 13% microangiopathy, 9% macroangiopathy, 11% cerebellar or brain stem infarction, 18% more than one cause and 18% no cause found. The patients with cardiogenic embolism showed significantly the highest scores on the stroke scale and the disability scale and had the shortest delay in admittance (57% were admitted within 3 hours). CONCLUSIONS: In a city hospital, cardiogenic embolism is the main cause of ischemic stroke. These patients suffer significantly the most severe neurologic deficits, dependence, and requirement of daily nursing care. These patients have the shortest delay in clinical admittance and the best chance of benefitting from acute therapy and early secondary prevention.

The GAA repeat expansion in intron 1 of the frataxin gene is related to the severity of cardiac manifestation in patients with Friedreich's ataxia.

Friedreich's ataxia is an autosomal recessive disorder characterized by spinocerebellar degeneration. It is caused by an unstable GAA trinucleotide repeat expansion (>120 repeats) in the first intron of the frataxin gene on chromosome 9 (9q13) in both alleles. Concentric left ventricular hypertrophy has been recognized as the major cardiac manifestation of Friedreich's ataxia. Our aim was to investigate the influence of the frataxin repeat length on cardiac hypertrophy in patients with Friedreich's ataxia and in patients with hypertrophic and dilated cardiomyopathy. Thirty-one patients with Friedreich's ataxia, 86 patients with hypertrophic cardiomyopathy, 134 patients with dilated cardiomyopathy, and 32 healthy individuals without cardiac disease were analysed by electrocardiography and 2D-M-mode echocardiography. Then, the size of the frataxin repeat was determined by polymerase chain reaction (PCR) and agarose gel electrophoresis. The number of GAA repeats in patients with hypertrophic and dilated cardiomyopathy was not different from the length in patients without cardiac disease (hypertrophic cardiomyopathy, 8+/-2 repeats on GAA 1 allele and 11+/-5 repeats on GAA 2 allele; dilated cardiomyopathy, 7+/-2 repeats on GAA 1 allele and 11+/-5 repeats on GAA 2 allele; Control, 9+/-1 repeats on GAA 1 allele and 12+/-6 repeats on GAA 2 allele). The septal and posterior wall thickness of these patients was not related to the GAA repeat length. All patients with Friedreich's ataxia had two enlarged alleles with a mean GAA repeat length of 757+/-316 and 1012+/-231, respectively. The lengths of both alleles were significantly greater than the lengths in the controls (P<0.0001), patients with hypertrophic cardiomyopathy (P<0.0001) and dilated cardiomyopathy (P<0.0001). A significant correlation was revealed between interventricular septal hypertrophy and frataxin repeat length in the smaller allele. Furthermore, the ratio of septal to posterior wall thickness was significantly correlated to GAA repeat size on the smaller allele. In conclusion, the size of the GAA repeat on the smaller allele in the frataxin gene is associated with the degree of left ventricular hypertrophy in patients with Friedreich's ataxia but is not related to the severity of hypertrophic cardiomyopathy.

SELEDO: a 5-year long-term trial on the effect of selegiline in early Parkinsonian patients treated with levodopa.

The SELEDO (from selegiline plus levodopa) study was carried out as a randomized, prospective, placebo-controlled, double- blind, multicenter long-term, 5-year trial to evaluate the possible advantages of combining selegiline and levodopa in the early treatment of Parkinson's disease. One-hundred-and-sixteen patients were randomized either to selegiline or placebo. Before starting the study medication, the levodopa dose was titrated to the individual requirements of each patient. The primary study end point (time when levodopa had to be increased by >50% of the titrated dose) was reached in 23 of 59 patients in the selegiline group and 26 of 48 patients in the placebo group. At the end of the 5 years' treatment period the rates derived from a life-table analysis were 50.4% in the selegiline group and 74.1% in the placebo group (P = 0.027, log-rank test). The median time to reach the primary end point was 4.9 years in the selegiline group and 2.6 years in the placebo group. In patients treated with selegiline, the mean levodopa dose changed only slightly over the 5 years of treatment compared to the initially titrated dose, but rose markedly in the placebo group, where the dose of levodopa had to be adjusted earlier than in the selegiline group. At the same time, the lower levodopa dosage in the selegiline group was accompanied by at least equal therapeutic efficacy (which is necessary for an unambiguous interpretation). Subgroup analyses showed greater benefit for selegiline treated) patients in the earlier stages. Long-term side effects appeared later in the selegiline group, although the difference was not significant. The early combination of selegiline and levodopa proved to be clearly superior to levodopa monotherapy.

[Lacunar brain infarct in carotid occlusion]. / Lakunärer Hirninfarkt bei Karotisverschluss.

The etiology of lacunar CCT lesions is controversial. We report a patient with recurrent ischemia in the territory of the right internal carotid artery. CCT initially showed a fresh non-lacunar lesion in the area of the basal ganglia and internal capsule. Subsequently, repeat CCT revealed a singular lacunar defect in the internal capsule. The Doppler sonographic investigations showed an internal carotid artery occlusion. This case supports the hypothesis that an embolic or hemodynamic etiology of the lacunae is possible.

[Cerebral infarct caused by compression of the carotid artery in an alcohol intoxicated patient]. / Hirninfarkt durch Kompression der Karotis bei einer Intoxizierten.

A 49 year-old woman developed a left sided hemiparesis with hemineglect after lying in an intoxicated condition for approximately half an hour with the right side of her neck on the metal edge of her bed. Dopplersonography of the intra- and extracranial vessels showed no abnormality of blood flow. On the right side of the neck a strangulation mark 8 cm of length was visible for several weeks. The CCT scan initially showed slight oedema. After 6 weeks changes corresponding to selective parenchymal necrosis were to be seen predominantly in the territory of the right middle cerebral artery.

Influence of additional information on interrater reliability in the neurologic examination.

We performed a routine neurologic examination on 200 patients who were reexamined by a second physician under three different conditions: (1) reexamination of only one item, (2) complete reexamination without knowledge of the patient's history and complaints, and (3) complete reexamination with such knowledge. We used changes in reliability as an index of the effect of additional information on basic data collection. Additional information increased reliability, indicating that diagnostic hypotheses obtained mainly from the history improve the examiner's precision. When a complete reexamination was performed with knowledge of the patient's history, reliability was substantial (kappa > 0.6). More experienced examiners do not perform the examination more reliably, but do utilize the patient's history differently.

Twenty-four-hour profiles of growth hormone, prolactin and cortisol in the chronic vegetative state.

The 24-h profiles of growth hormone (GH), prolactin (PRL) and cortisol were obtained from 11 patients in the chronic vegetative state in order to gain more insight into the neuroendocrine alterations caused by widespread suprahypothalamic brain damage. Age and sex-matched normal subjects served as a control group. Patients had fewer high-amplitude GH peaks (greater than 20 mU/l: 6 peaks/24 h vs 21 peaks/24-h in controls) and a (non-significant) tendency towards higher basal GH concentrations. PRL concentrations were higher in patients (296 +/- 212 (SD) vs 120 +/- 28 mU/l). Cosinor analysis also showed that 24-h rhythmicity was preserved, but acrophases were more dispersed. A nocturnal PRL acrophase occurred in only three of 11 patients but in 10 of 11 control subjects. The number of PRL peaks was the same in patients and controls. Cortisol concentrations were also higher in patients (298.3 +/- 114.6 vs 193.6 +/- 97.4 nmol/l) with a preserved circadian rhythm. The acrophases, however, were likewise more dispersed. There was no difference in the number of cortisol peaks between patients and controls, but the mean peak duration was shorter in patients (75.4 +/- 28.1 vs 109.5 +/- 28.2 min). The stage of remission was negatively correlated with the 24-h mean and the mean peak amplitude of PRL. No patient showed a normal organization of sleep stages. On visual analysis there was no apparent association between EEG patterns and hormonal parameters. These results suggest that the endocrine hypothalamus is essentially intact in the chronic vegetative state. The observed changes may be due to an altered input from extrahypothalamic brain structures.

[Ulcero-mutilating acro-osteopathy in hereditary neuropathies. Differential diagnosis and pathogenesis]. / Akroosteopathia ulcero-mutilans bei hereditären Neuropathien. Differentialdiagnostische und pathogenetische Aspekte.

Two patients, aged 51 and 70 years, had indolent ulcerations at the sole of the foot with destructive osteolysis in the bones of the feet, lesions characteristic of mutilating ulcerative acro-osteopathy. Patient 1, who had pes cavus, developed ulcerations on the balls of the feet along the second metatarsal bones, and patient 2 with pes equinovarus developed ulcers in the area of the calcaneus. Sock-like hypesthesia/hypalgesia from the toes to the ankles was present in both patients, and electrophysiological tests confirmed the presence of axonal sensory-motor neuropathy. Diabetes mellitus and alcohol abuse was excluded in both patient. Clinical findings, history and neurological disturbances in both patients identified the disease as hereditary sensory neuropathy (type I).

Laminin immunostaining in hyperplastic, dysplastic, and neoplastic lesions of the endometrium and uterine cervix.

The distribution of basement membrane laminin was investigated in normal endometrium and cervix, as well as in a variety of non-neoplastic and neoplastic lesions of these tissues. Normal epithelial structures were surrounded by a generally intact basement membrane. Minor breaks in continuity and alterations of linearity were seen in association with inflammation and stromal fibrosis. Metaplasias, atypical hyperplasias, dysplasias, and in situ carcinomas characteristically rested on an essentially intact basement membrane, although minor disruptions were seen, occasionally in association with inflammation. Superficially and deeply invasive adenocarcinomas and squamous carcinomas exhibited impaired laminin production. In well differentiated carcinomas of the endometrium and cervix, an intact basement membrane was often seen around infiltrating glandular and squamous elements, although focal reduplications and small disruptions were seen in all cases. In high-grade carcinomas, considerable impairment of basement membrane integrity was seen, but immunostainable laminin was still evident, at least focally, in all cases. The findings confirm that endometrial and cervical carcinomas, even those of high histologic grade, are capable of basement membrane production.

Effect of hypoglycaemia, TRH and levodopa on plasma growth hormone, prolactin, thyrotropin and cortisol in Parkinson's disease before and during therapy.

Thirteen drug-free and not severely affected patients with idiopathic Parkinson's disease underwent an insulin-hypoglycaemia test, a TRH test and a levodopa test. The responses of growth hormone, prolactin, cortisol and thyrotropin were measured, and retested under stable therapy with levodopa and benserazide. Mean basal and stimulated hormonal concentrations were in the normal range before and during therapy. Minor abnormalities were observed in individual cases, but did not indicate a hypothalamic dopamine deficit.

Long-term effects of spinal cord stimulation in chronic pain syndromes.

A total of 50 patients with chronic pain syndromes were selected for treatment with spinal cord stimulation. Correct positioning of electrodes was obtained in 44 patients, leading to an initial alleviation of pain in 25 patients. In 6 patients, electrodes (though still effective in 4) had to be removed because of surgical complications within the first 5 months of use. Only 8 patients had at least some beneficial effect lasting for more than 3 years. The long-term results in patients with more severe psychological disturbances were no worse than those of the other patients.