[Triple arthrodesis in treatment of degenerative hindfoot deformities: clinical, radiological and pedobarographic results]. / Die Triple-Arthrodese in der Therapie degenerativer Rückfussfehlstellungen: Klinische, radiologische und pedobarografische Resultate.

INTRODUCTION: Triple arthrodesis is performed to reconstruct a painless plantigrad foot in cases of fixed and painful hindfoot deformity. Mid-term results of our patients after triple arthrodesis concerning clinical and functional as well as radiological outcome were assessed in this examination. PATIENTS AND METHOD: In the retrospective study, 24 feet in 20 patients with triple arthrodesis after degenerative hindfoot malposition were examined. Sampling was done consecutively. The average age of the patients was 62.8 years (40-79), mean follow-up-period was 21.5 months. The patients were examined using the AOFAS score, assessment of subjective satisfaction and X-rays. In addition, the position of the foot was analysed using dynamic pedobarography and gait analysis. RESULTS: In the examined population, the mean AOFAS score was 74 (+/- 12.41) out of 94 points, the improvement averaged 51 (+/- 14.72) points. 75 % of the patients had no or only slight pain, 8 % reported limitations in everyday life. Mobility of the ankle was more than 30 degrees for 9 patients (38 %) and between 15 and 30 degrees for 13 patients (54 %). 23 patients out of 24 were satisfied with the result of the surgery. In most cases, dynamic pedobarography showed a rather physiological pattern of plantar pressure distribution with increased pressure remaining in the midfoot area. Valgus position of the hindfoot was 2.8 degrees (+/- 2.9 degrees), gait analysis showed very good results in 11 cases and good results in 13 cases. Among the examined patients, two cases of wound healing disorders appeared, and one non-union of the talonavicular joint which had to be reoperated. In two cases, clinically relevant degenerative changes of the ankle were diagnosed. CONCLUSION: Triple arthrodesis using internal fixation followed by sufficient immobilisation is a good and reliable technique for the correction of fixed hindfoot deformities. A very high level of patient satisfaction and a good clinical outcome can be achieved. Plantar pressure distribution can be reconstructed to a satisfactory extent. Due to the relatively high rate of degenerative arthritis in adjacent joints, the decision upon performing a triple arthrodesis should be considered carefully. Clinical and radiological integrity of these joints are required.

The mirror world of motor inhibition: the alien hand syndrome in chronic stroke.

Alien hand syndrome (AHS) is rare, but important due to its disabling impact on everyday life. The determining characteristic of AHS is intermanual conflict, a type of inhibitory motor behaviour that occurs against willed action. Its components have previously been described as single case reports, but not as a systematic study. This review includes eight chronic cases, all of which are due to infarcts of the anterior cerebral artery. Clinical investigation included testing of motor behaviour related to everyday activities, such as tying shoelaces, lighting a candle and other bimanual tasks. Video-analysis showed that conflicting behaviour occurs in two distinct forms. One consists of interfering, rudimentary, hesitant and repetitive movements of the (alien) hand, often initiated by movements of the other hand. In some instances, disturbance of ongoing action is seen as spacious, ballistic-like extensions of the whole arm. This was most prevalent in three of the eight cases. In one patient, it was also seen as conflict with both feet (eg, when putting on slippers) or as a conflict of intentions (eg, when planning to enter a room). The other form consisted of massive groping and grasping behaviour as the most dominant features, such as a "tug of war between hands", and was seen in five patients. Avoidance behaviour included sitting on the affected arm, holding it under the table or keeping objects out of reach. Enforcement of such strategies was used for rehabilitation and--although beneficial in the training sessions--carried over very little to everyday life. All cases had two distinct brain lesions, one in the genu or anterior rostrum of the corpus callosum and one in the contralateral frontomedial cortical and subcortical region. Chronic AHS is the only clinical syndrome that shows complex inhibitory motor behaviour in a more or less pure form because it has become detached from the control of motor planning and execution. It can best be understood as sequences of complex inhibitory motor programmes that have become isolated from normal motor planning, which usually suppresses them via the contralateral cortico-subcortical prefrontal circuits and the corpus callosum. Thus, the mirror world of complex motor inhibition becomes clinically visible in such patients.

Relation of cardiac abnormalities and CTG-repeat size in myotonic dystrophy.

It is unclear if the severity of cardiac involvement in patients with myotonic dystrophy (MD) is related to the size of the CTG-repeat expansion. This open, uncontrolled, observational, prospective study aimed to find out if there is a relation between the severity of cardiac involvement in MD and the CTG-repeat size. In 21 patients with MD, (8 women, 13 men, aged 11-88 years) a detailed cardiologic examination, including history, clinical examination, electrocardiography (ECG), transthoracic echocardiography and ambulatory 24-h ECG, was carried out and cardiac involvement was assessed according to a previously described scoring system. Additionally, the CTG-repeat size was determined from nuclear DNA of blood leukocytes. The correlation between the CTG-repeat size and the mean heart rate, PQ-interval, QTc-interval, fractional shortening, left ventricular enddiastolic diameter, septal thickness, posterior wall thickness, mean heart rate on 24-h ECG and cardiac involvement score was r=0.47, r=0.086, r=0.11, r=-0.27, r=-0.34, r=-0.06, r=-0.12, r=0.16 and r=0.09 (all p>0.05), respectively. In patients 21-30, 31-40 and 41-50 years of age, cardiac involvement increased with increasing CTG-repeat size. In younger patients, the number of CTG-repeats needed to develop a reasonable cardiac involvement was higher than in older patients. Depending on age, cardiac involvement increases with increasing CTG-repeat size obtained from blood leukocytes in patients with MD.

[Time delays in admission to a Stroke Unit and emergency treatment of patients with ischemic stroke]. / Zeitliche Verzögerungen bei der Zuweisung und Akutbehandlung von ischämischen Insulten an einer Stroke Unit.

BACKGROUND AND OBJECTIVE: This observational study describes the time delays involved in the emergency treatment of acute strokes admitted for treatment in the acute stroke unit of the Landesnervenklinik Gugging, Austria. The aim was to define avoidable delays in the prehospital and intrahospital phase. PATIENTS AND METHODS: 261 stroke patients were included consecutively within the one-year study period (September 1996 until September 1997). Minute-by-minute reconstruction of events starting from the first symptoms to the first therapeutic application within the stroke unit was performed for every stroke patient by a neurologist during or immediately after treatment. RESULTS: Time delays are predominantly in the prehospital phase. Only 20.5% are admitted within 120 minutes after noticing first symptoms. Public registration time was 42 +/- 212 minutes. In the intrahospital phase, the mean door-to-drug-time was 50 +/- 28 minutes (including CT). In 25% of the patients, adequate treatment of acute stroke was started within 35 minutes after the patient's arriving at the hospital. In 92% cerebral computed tomography was performed before treatment was started. CONCLUSIONS: This study shows the necessity for continuing efforts to increase public awareness for immediate hospitalisation after stroke in order to achieve a higher rate of urgent and direct admission to a stroke unit.

[Medial medullary infarct]. / Der mediale medulläre Infarkt.

Medial medullary infarct is a rare type of brain stem infarction first described in 1908. It was only since the broad use of MRI that an accurate clinical topographical correlation could be documented in survivors. We observed two patients; one of them had an anteromedial unilateral infarction in the upper medulla, and the clinical picture was characterized by contralateral hemiparesis, facial weakness, dysarthria and palatal weakness. The outcome was good. The second patient however showed a severe tetraparesis with only minor brain stem signs, secondary to bilateral anteromedian infarction with a large craniocaudal extension. He survived with severe residual impairment. The most likely cause of infarction was arteriosclerosis of the A. vertebralis and A. spinalis anterior, respectively. These two different cases suggest that the medial medullary syndrome is heterogeneous comprising small unilateral infarctions with mild clinical signs as well as large bilateral infarctions with a poor outcome.

Parkinsonism and dystonia in central pontine and extrapontine myelinolysis.

Parkinsonism as well as dystonic signs are rarely seen in central pontine myelinolysis and extrapontine myelinolysis. A 51 year old woman developed central pontine myelinolysis and extrapontine myelinolysis with parkinsonism after severe vomiting which followed alcohol and drug intake, even though marked hyponatraemia had been corrected gradually over six days. Parkinsonism resolved four months after onset, but she then exhibited persistent retrocollis, spasmodic dysphonia, and focal dystonia of her left hand. Although the medical literature documents three similar patients, this patient is different as dystonic symptoms only developed four months after parkinsonian signs had resolved.

Silent brain infarcts and transient ischemic attacks. A three-year study of first-ever ischemic stroke patients: the Klosterneuburg Stroke Data Bank.

BACKGROUND AND PURPOSE: We undertook to study the clinical relevance of silent strokes and history of transient ischemic attacks (TIAs) and their individual and combined effects on outcome variables of neurological and epidemiological interest in first-ever stroke patients. METHODS: We performed univariate and multivariate analyses of data prospectively collected in the Klosterneuburg Stroke Data Bank, a hospital-based registry in Austria that includes a 3-year follow-up program. RESULTS: Of 728 patients (mean age, 68 +/- 10 years) with a first-ever ischemic stroke, 110 (15%) had had a previous TIA, and 66/618 (11%) patients did not have a history of TIA but showed evidence of silent brain infarct on CT. Outcome variables of neurological interest were not significantly different between groups, including time between stroke and study entry, activities of daily living status at first presentation, median time of hospitalization, 30-day mortality, or 3-year mortality. Univariate analyses of epidemiologically important risk factors showed either history of TIA or evidence of silent infarct to be more frequently associated with hypertension (P = .007). Cox models of survival showed that neither history of TIA nor evidence of silent infarct were significantly associated with an increase in 3-year mortality. CONCLUSIONS: Over a period of 3 years, neither history of TIA nor evidence of silent infarct diagnosed at the time of the presenting major stroke in first-ever ischemic stroke patients exert an important influence on neurological or epidemiological outcome variables.

GM 1 antibodies in Guillain-Barré syndrome: isotypes, course and clinical outcome.

IgA, IgG, and IgM antibodies against ganglioside GM 1 were investigated in sera of 15 patients with Guillain-Barré syndrome (GBS), as compared with healthy controls. Significantly raised titers were found in 4 patients who showed persisting weakness due to muscle wasting (3 patients) or due to the development of a chronic relapsing form of GBS (1 patient) after a follow up period of 1 year. By contrast, only 1 out of 11 anti-GM 1 seronegative patients showed persisting muscle wasting. In 1 patient with GBS following Campylobacter jejuni enteritis an IgA anti-GM 1 immune response was found to predominate, suggesting that this immune response may be due to crossreactivity between GM 1 and Campylobacter antigens. We conclude that elevated antibody titers against ganglioside GM 1 may be a valuable marker to predict poor motor recovery in GBS.

Silent cerebral infarction in stroke patients: Results from the klosterneuburg stroke data bank (Austria).

The incidence, topology, and risks of clinically silent cerebral ischemia were studied in a series of stroke patients entering the Klosterneuburg Stroke Data Bank, a prospective single-center stroke registry in Lower Austria that was established in 1988. Among 462 first-ever stroke patients, 340 (74%) showed only one lesion, which corresponded to the syndrome of their presenting stroke. However, 57 (12%) patients either showed an additional or a noncorresponding lesion, or both. Most silent strokes in our study were singular, deep, small infarctions (52%) by their location known to be asymptomatic, whereas eight additional cases with cortical involvement and one deep, large infarction could have been either unreported or largely asymptomatic. No significant difference was found for age, sex, or the hemispheric side of any lesion type (deep, small, or cortical), nor for the etiology of the presenting stroke. Silent cerebral infarctions were not associated with known additional or disproportional risk factors. The differing incidences and risks of silent stroke known from previous studies probably are not only due to the differing accuracy of neuroimaging techniques to detect small ischemic lesions but also to differences in awareness of unspecific and transient symptoms. Therefore, a continuum between clinically silent infarcts, unspecific symptoms, and short-lasting transient ischemia must be acknowledged.

Stroke subtype is an age-independent predictor of first-year survival.

The short-term outcome after acute stroke is known to be strongly dependent on stroke subtype, especially favoring patients having suffered a lacunar stroke. The value of stroke subtypes as long-term predictors of survival has not been firmly established. We therefore examined the 1-year survival from acute stroke in the Klosterneuburg Stroke Data Bank, which since 1988 systematically collects data on acute stroke patients treated in one center in Lower Austria. The cumulative survival of 398 consecutive patients (mean age 67.7 +/- SD 11.6) 1 year after a first-ever stroke was not only adversely related to age but also appeared to be age independent when compared according to stroke subtypes. The cumulative survival rates (CSR) ranged from 88.7 [95% confidence interval (CI): 82-92] for lacunar strokes (n = 107) to 68.1 (CI: 50-79) for atherothrombotic strokes (n = 69). Cardiogenic embolism (n = 68) showed a CSR of 72.1 (CI: 61-83) similar to the group of cryptogenic strokes (n = 121, CSR: 74.4, CI: 67-82). The CSR for primary intracerebral hemorrhage (n = 33) was 68.8 (CI: 53-85). It is concluded that the comparatively favorable prognosis of lacunar strokes must be recognized in trials designed to evaluate efficacy in terms of 1-year mortality. Furthermore, as cryptogenic stroke has an almost identical survival rate when compared to strokes caused by cardiogenic embolism it may be assumed that a considerable number of strokes for which no cause can be found are, in fact, cardiogenic embolic.

[Critical illness polyneuropathy: clinical aspects and long-term outcome]. / Critical Illness Polyneuropathie: Klinik und Langzeitergebnisse.

Patients treated in intensive care units may develop a primary axonal form of polyneuropathy complicating sepsis and multiple organ failure more frequently than previously assumed. This critical illness polyneuropathy causes difficulty in weaning patients from the ventilator and delays further recovery and mobilisation. Over a period of two years we have treated five patients with flaccid tetra- or paraparesis. Recovery of motor function was largely satisfactory, but a long rehabilitation process was necessary. If attention were paid to detecting this disease in the early stages of intensive care neurorehabilitation might be facilitated. Hence, electrophysiological tests should be performed as soon as possible. The clinical outcome was markedly influenced by long-lasting neuropsychological disturbances in three of the five patients as well as by other complications such as joint contractures.

Pigmentary type of orthochromatic leukodystrophy with early onset and protracted course.

The pigmentary type of orthochromatic leukodystrophy (OLD) is a rare disorder in adults; only one questionable childhood case has been observed. We report the sporadic case of a male aged 26 years with early onset and protracted course. He presented retarded motor development from birth with ataxic gait and, at age 13 years, developed progressive mental and neurologic deterioration with tetraparesis, ataxia and seizures and died in a disabled, mute state. Repeated CT scans showed progressive diffuse cerebral atrophy and low density of the hemispheric white matter. Autopsy revealed OLD with pigmented macrophages and glial cells, ultrastructurally showing storage of lipofuscin and ceroid with multilamellar bodies or finger-print profiles. Abnormal cytoplasmic inclusions in reduced oligodendroglial cells suggest demyelination due to a primary defect of oligodendroglia in this rare disorder.

[Recurrent and relapsing course of borreliosis of the nervous system]. / Zur rezidivierenden und schubhaft verlaufenden Borreliose des Nervensystems.

The clinical variability of chronic infections due to Borrelia burgdorferi is greater than previously thought. Three personal cases are presented in an overview, together with cases from the literature. Chronic spastic para- and quadriparesis, transverse myelitis and recurrent hemiparesis have been noted in such cases. Frequently, there is additional involvement of the cranial nerves. Chronic polyneuritis, mononeuritis multiplex, as well as plexus neuritis can also occur. Psychiatric manifestations can at times be predominant. Their spectrum ranges from agitated depressive states with suicidal ideas to the clinical picture of dementia. Due to the high percentage of positive antibody titre reactions in the normal population, reliable evidence of a chronic infection of the nervous system is based on the examination of the cerebrospinal fluid. Lesions seen on CT and MRI are mostly uncharacteristic and bear no clear-cut relationship to the disease. Neurological signs and symptoms can be improved by antibiotic treatment.

[Neurotoxicity in long-term lithium therapy: an electroneurographic study]. / Neurotoxizität bei Langzeit-Lithiumtherapie: eine elektroneurographische Studie.

In a series of 16 patients on long-term lithium therapy pathological changes were found in peripheral nerve conduction. Such changes correlated with the duration of lithium therapy (mean: 8.3 years, range: 3-15 years; p less than 0.05). No patient showed clinical signs of polyneuropathy or any other disease known to be frequently associated with peripheral nerve lesions. Compared with an age-matched normal control group (n = 27) the nerve conduction velocity of the sural nerve was reduced (p less than 0.05), for which a chronic neurotoxic effect is considered to be the most probable mechanism.

HLA-antigen distribution in seminoma, HCG-positive seminoma and non-seminomatous tumours of the testis.

Histocompatibility antigens play a certain role in the development of testicular tumours. 151 patients with testicular cancer (86 non-seminomatous germ cell tumours--NSGCT--and 65 pure seminoma) were typed for the HLA-antigens of the A, B, C and DR locus. 24 patients of the pure seminoma group and 50 patients of the NSGCT group had an elevated serum HCG level preoperatively. The antigen DR-5 was elevated in the seminoma group whereas the incidence of B-13 was increased in the NSGCT group. In terms of antigen distribution HCG-positive seminoma resembles seminomatous tumours rather than NSGCT.

Disseminated superficial "actinic" porokeratosis.

The dramatic therapeutic response of disseminated superficial actinic porokeratosis (DSAP) to retinoid plus psoralens with ultraviolet A prompted a review of clinical, histologic, and etiologic data of all of the DSAP cases available in the English and German literature. The review showed that many case reports lack adequate documentation to confirm actinic induction. More than one third of the patients have observed no exacerbations during the summer. Histologic damage after long-term ultraviolet (UV) exposure could not be observed in every case. In addition, cases do exist in which lesions are distributed mainly in non-UV-exposed skin. These data cause doubt about the importance of actinic induction of DSAP.