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Paul Ferdinand Schilder was born in Vienna in 1886 and died in New York in 1940. He is nowadays remembered predominantly for his contributions to modern psychiatry and psychotherapy; however, he was also a neurologist and neuroscientist and in particular in his early years, he researched and published on neuropathological topics. This paper focuses on his scientific work during his years in Middle Germany (1909-1914), where he worked with Gabriel Anton in Halle and Paul Flechsig in Leipzig. During those years, he laid the foundations for his definition, clinical classification and differentiation of encephalitis periaxialis diffusa. Today, this inflammatory brain disease is known as Schilder's disease and is of some importance as a rare differential diagnosis of multiple sclerosis (MS), especially in children. Schilder's reflections and findings were based on his scrupulous and detailed analysis of only a few medical histories, which also comprised histological neuropathological examinations, as well as on his extensive and critical review of the relevant literature of the time. His aim was to differentiate encephalitis periaxialis diffusa from brain tumors, MS and Heubner's diffuse sclerosis. Schilder's scientific achievement, made in relatively young years, is still impressive even to the present day due do its thoroughness and accuracy as well as the enormous workload and ambition it required. Even though ambitious, Schilder was always prepared to critically review his own ideas.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary stroke disease linked to chromosome 19 q 12. We report on two families affected by this disease, which is characterised by recurrent subcortical ischaemic strokes, progressive pseudobulbar palsy, and presenile dementia. Neuroimaging revealed multiple deep infarcts and diffuse leukoencephalopathy. Diagnosis of CADASIL based on the typical clinical feature without vascular risk factors, the neuroradiological signs of extensive leukoencephalopathy, and the autosomal dominant pattern of inheritance. CADASIL might be an underestimated cause of familial stroke and should be considered in the differential diagnosis of hereditary stroke.

The surgical treatment of chronic epilepsies is increasing rapidly. A review of 279 surgical specimens of patients with chronic pharmaco-resistant epilepsies from the University of Bonn Medical Centre revealed specific histopathological findings in 89% of all well-preserved specimens. The most frequent focal lesions were low-grade brain tumours such as gangliogliomas, pilocytic astrocytomas and dysembryoplastic neuroepithelial tumours. Other common findings were glioneuronal and vascular malformations and Ammon's horn sclerosis. This suggests that chronic intractable epilepsies are frequently caused by structural alterations.

Adrenoleucodystrophy (ALD) is an uncommon, sex-linked disease, characterised by the accumulation of very long-chain fatty acids in various tissues. There is a great predilection for young boys between the age of three and ten years. The disease ends fatally after a clinical course of several years. The clinical and MR findings of a child with typical ALD are presented. T2-weighted MR demonstrates confluent, symmetrical, hyperintense lesions of the occipital white matter bilaterally. After the application of Gd-DTPA, contrast enhancement is seen in the margins of the lesions indicating active demyelination. MR is recommended as imaging modality of choice in patients with suspected or confirmed diagnosis of adrenoleucodystrophy.

Four cases with the clinical diagnosis of chronic MS are presented, which, histologically, show large solitary demyelinated and gliotic areas involving almost the entire spinal cord over 12-25 segments as well as the optic chiasm. The abortive form of the neuromyelitis optical syndrome in MS is discussed. The additional involvement of the cerebral hemispheres suggests that the clinical and histological picture described is related to Schilder's disease.

An unusual case of neuraxonal dystrophy in a 24-year-old man has been described. The condition started at the age of 10 years with motoric disturbances, later the patient developed spastic quadruparesis and deep dementia in last two years. The disease appears to be of genetic origin. The morphology is characterized by the generalized presence of spheroids in the central nervous system. Additional findings of status pigmentosus and dysmyelinisatus do not exclude the case from being labelled neuraxonal dystrophy.

Adrenoleukodystrophy (ALD) is characterized by adrenal cortical insufficiency and progressive demyelination of the central nervous system with lethal outcome in childhood. Adrenomyeloneuropathy (AMN) represents a clinical variant of ALD with later manifestation during adolescence. The neurological symptoms of this form are slowly progressive spastic paraparesis and peripheral neuropathy. Other intermediate forms, which might be classified as adrenoleukomyeloneuropathy (ALMN), possibly represent an overlap of the two conditions. Heterozygote carriers of the X-linked disease may also show neurological symptoms, such as spastic paraparesis and peripheral neuropathy. Biochemically, all these X-linked forms of ALD/AMN are characterized by an accumulation of very-long-chain fatty acids in various tissues and body fluids indicating an impaired peroxisomal metabolism. In this study two families with ALD are presented. The two forms of ALD and AMN were observed in different members of the families including a heterozygote female carrier with neurological symptoms. The different clinical syndromes, the biochemical and genetic basis, and new therapeutical strategies are discussed.

In 6 patients with acute or exacerbating demyelinating disease and in 2 cases suffering from adrenoleucodystrophy (Schilder's disease) brain scintigraphy revealed areas with increased isotope uptake. The pathological foci were verified by autopsy in 4 cases and by inspection in 1. These foci are difficult to distinguish from other localized brain diseases causing increased isotope uptake; in addition to the clinical course and to spinal fluid abnormalities reversibility of the scintigraphic lesions indicate demyelinating diseases. The typical pattern of symmetrical lesions within the white matter and the progression of abnormalities from the occipital to the frontal lobe speak in favor of Schilder's disease.

The diagnosis of adrenoleukodystrophy (ALD) in two brothers was confirmed by the analysis of long-chain fatty acids in cultured skin fibroblasts. The 23 year old brother was treated for Addison's disease at the age of 7 years. His first symptoms of ALD developed at the age of 22. These were lack of concentration and compulsive disorder. The younger brother was noted to show behavioural changes and a decreased performance at school at age 9. The disease then progressed rapidly. Variability and diagnostic procedures are discussed in this report.

We report on a male infant with spongy degeneration of the central nervous system (van Bogaert and Bertrand type), who died at the age of 7 months. The clinic as well as the diagnostic procedure are described, especially the importance of cranial computerized tomography for the diagnosis.

This article presents the clinical and pathological history of a 10-year-old boy with adrenoleukodystrophy as well as a tabular survey of 17 additional cases of which the necropsy records were published in the literature. So far as the case of adrenoleukodystrophy directly observed by the authors themselves is concerned, commencement of the disease was in the seventh year of life, with symptoms of Addison's disease and diffuse inflammatory cerebral sclerosis being observed at about the same time. The cerebral disease manifested itself as a progressive pseudobulbar and pyramidal tract symptomatology and as a progressive cerebral psychosyndrome reminiscent of an endocrinal psychosyndrome. Morphologically, the adrenoleukodystrophy was characterized by severe dystrophy of the cortex of the suprarenal gland and the brain are considered the points of action of a still unknown noxa.e suprarenal gland as well as by extensive sudanophilic processes of decomposition in the cerebromedullary substance, putamen, pallidum, and thalamus. Striated cells and cytoplasmic filamentous inclusions observed in the brain and suprarenal glands in a number of cases reported in the literature were not found by the present authors. The cortex of th

The brain of a 21-year-old man with imbecility and spastic tetraplegia since early childhood showed a diffuse sclerosis of the cerebral white matter and symmetrical degeneration of the thalamus and olivocerebellar system. The spinal nerve roots as well as proximal branches of the spinal nerves were hypertrophic and there was seen a marked fibrosis with some onion-bulb formations of Schwann cells. In spite of a possible relation to an unknown infection at the age of 3 months this case could preferably be regarded as a peculiar form of the combined system degeneration in the early childhood, including the cerebral white matter and peripheral nerves. An uncle of the patient had died of a similar nervous disease at the age of 20 years.

Geriatric rehabilitation is described according to the theory of Steinmann and is divided into three categories. Firstly, into the actual preventive rehabilitation of the outpatient with the aim of slowing down, possibly even stabilizing or retarding partly normal, partly pathological changes occurring as a function of the aging process. The old person who is not confined to an institution should thus be given an opportunity to lead an active life inspite of certain age related infirmities. Secondly, the general rehabilitation of the sick, comprising the treatment of age illnesses by means of medication and what could be described as "activating care or therapy". Thirdly, the specific rehabilitation which deals with these old age diseases through sophisticated and expensive team in a highly specialized clinic. The approach of the therapeutic team of a geriatric clinic ist described on the basis of the example of the treatment of hemiplegic patients.

A method for quantifying the CSF compartments in cranial computer tomograms is described. The method is based on an analysis of the histogram curve, the proportion of area and thickness of the liquor spaces being related to the total area below the curve. In order to isolate the skull contents, a CT masking technique is used. The practicability and usefulness of the method in clinical practice was tested in 59 normal patients and in eleven patients with rapidly progressing brain atrophy.

A report on clinical course and morphological findings of a case of adrenoleukodystrophy in a 10-years-old boy is given. The brain showed the typical changes of a sudanophilic, inflammatory leukodystrophy in all lobes. Pathognomic intracytoplasmatic inclusions in the perivascular macrophages were found by electron microscopic examination. The adrenal glands showed a severe atrophy, but it was impossible to detect typical cytolpasmatic inclusions within the adrenocortical cells. By thin-layer chromatography an accumulation of cholesterol esters with very long chain fatty acids was found in the demyelinated areas. Findings diagnostic facilities and theories of pathogenesis are discussed.

After 5 years of cyclic vomiting an 8 year old boy was presented with coma and hyponatremia. ACTH and renin plasma concentrations were elevated, cortisol concentrations did not rise after ACTH-stimulation. Behavioural abnormalities including secondary enuresis and dysarthria drew attention to the possibility of an association of adrenal insufficiency with leucodystrophy. NMR tomography of the brain showed symmetrical demyelinated areas in the parieto-occipital regions. Very long chain fatty acids were elevated. The mother showed discrete neurological symptoms and elevated long chain fatty acids. Cyclic vomiting might suggest adrenoleukodystrophy.

In the case of many alcoholics there is a risk of misuse of, and dependence on, sedative drugs, including clomethiazole. Used strictly in its proper indication and in the proper manner, the risk of dependence on clomethiazole is probably rather small. Proper indication and use in the case of alcoholics means treatment limited in time to the withdrawal period, i.e. as a rule not longer than six to seven days. Non-alcoholic/elderly patients suffering from cerebral sclerosis do not tend to misuse drugs; unlike alcoholics, there is therefore in the case of such elderly patients in general no risk of the development of dependence.

In a 31 year old patient with adrenocortical gland insufficiency and minor clinical signs of involvement of peripheral and central nervous system an adrenoleucodystrophy was diagnosed, based upon the characteristic elevation of hexacosanoic acid in plasma. The motor and sensory nerve conduction velocities in upper and lower extremities were markedly reduced; in accordance, the somatosensory evoked potentials showed distinct reduction in the peripheral part. In contrast, the interpeak latencies N 20 - NSEP 3 (median nerve) and N 22 - P 40 (tibial nerve) were in the upper normal range, so that a damage of the central sensory system could not be evaluated. The visual and brainstem acoustic evoked potentials were a little prolonged. The problems in differential diagnosis of this form of adrenoleucodystrophy, which especially involves the peripheral nervous system, are discussed.