Hereditary cerebral haemorrhage with amyloidosis, Dutch type (HCHWA-D): clinicopathological studies.

Clinical and neuropathological findings are reported in 63 patients with hereditary cerebral haemorrhage with amyloid angiopathy. Patients had mostly recurrent strokes, and at least 80% of these were haemorrhages. Almost a third of the patients died within a year of their first and only recorded haemorrhage, half of them within two weeks.

Handedness and progressive hydrocephalus in spina bifida patients.

Hand preference and functional hand-dominance of 45 spina bifida patients (aged 13 to 25 years) were assessed by means of self-reports on an ordinal scale and a tapping task. Non-right-handedness was more frequent among patients with accompanying progressive hydrocephalus. Left-hand preference was significantly more frequent in this group than in patients without shunts.

The smile in anencephalic infants.

During the period 1947-1959 twelve new-born mero-anencephalics were observed, who survived for more than 1 day after birth. Two even lived for 20 and 237 days, respectively. In 4 of the 12 children studied touching or slight pressure, and also faradic stimulation of various spots of the cerebro-vascular area were invariably followed by a facial expression which was very similar to smiling.

Expression of a normal and variant Alzheimer's beta-protein gene in amyloid of hereditary cerebral hemorrhage, Dutch type: DNA and protein diagnostic assays.

Amyloid fibrils deposited in cerebral vessel walls in Dutch patients with hereditary cerebral hemorrhage with amyloidosis (HCHWA-D) are formed by polymerization of a 39-residue peptide similar to the beta-protein of Alzheimer's disease, Down syndrome, sporadic cerebral amyloid angiopathy and normal aging. Sequence analysis of genomic DNA in HCHWA-D patients demonstrated a point mutation, cytosine for guanine at position 1852 of the precursor beta-protein gene, which causes a single amino acid substitution (glutamine for glutamic acid) corresponding to position 22 of the amyloid protein. The normal allele was also present in these patients.

Mutation of the Alzheimer's disease amyloid gene in hereditary cerebral hemorrhage, Dutch type.

An amyloid protein that precipitates in the cerebral vessel walls of Dutch patients with hereditary cerebral hemorrhage with amyloidosis is similar to the amyloid protein in vessel walls and senile plaques in brains of patients with Alzheimer's disease, Down syndrome, and sporadic cerebral amyloid angiopathy. Cloning and sequencing of the two exons that encode the amyloid protein from two patients with this amyloidosis revealed a cytosine-to-guanine transversion, a mutation that caused a single amino acid substitution (glutamine instead of glutamic acid) at position 22 of the amyloid protein. The mutation may account for the deposition of this amyloid protein in the cerebral vessel walls of these patients, leading to cerebral hemorrhages and premature death.

Does the artery of Adamkiewicz exist in the albino rat?

The arterial supply of the spinal cord of the rat in the thoracolumbar region was studied in 26 Wistar albino rats. Contrary to the situation in man, and in variance with earlier reports for the rat, we did not observe a single large artery at the thoracolumbar level supplying a large part of the spinal cord. The anterior spinal artery at this level seemed to be adequately supplied by three to five principal arteries.

Beta-protein deposition: a pathogenetic link between Alzheimer's disease and cerebral amyloid angiopathies.

Cerebral amyloid angiopathy (CAA) refers to a group of hereditary (hereditary cerebral hemorrhage with amyloidosis, HCHWA and sporadic (SCAA) disorders characterized by amyloid fibril deposition restricted to the leptomeningeal and cortical vasculature leading to recurrent hemorrhagic and/or ischemic accidents. On clinical and biochemical grounds, two forms of HCHWA can be distinguished. The amyloid subunit of the HCHWA of Icelandic origin is related to Cystatin C, while amyloid from patients of Dutch origin (HCHWA-D) is related to the beta-protein (or A4), the main component of vascular and plaque core amyloid in Alzheimer's disease (AD) and Down's syndrome (DS) [corrected].

Hereditary cerebral haemorrhage caused by cortical amyloid angiopathy.

This article describes 136 patients with hereditary cerebral haemorrhages; all patients belonged to families (originally) resident in Katwijk (The Netherlands). Cases of hereditary cerebral haemorrhage have also been reported in NW-Iceland, and in the Dutch coastal village of Scheveningen. Katwijk is a Dutch fishing-village, located 20 miles north of Scheveningen.

Different processing of Alzheimer's beta-protein precursor in the vessel wall of patients with hereditary cerebral hemorrhage with amyloidosis-Dutch type.

Hereditary cerebral hemorrhage with amyloidosis in patients of Dutch origin is an autosomal-dominant type of amyloidosis restricted to the small vasculature of the brain and clinically characterized by recurrent strokes. Amyloid fibrils from the leptomeninges of two patients were isolated and the primary structure determined. The complete sequence of the amyloid protein shows homology to the vascular (beta-protein) and plaque amyloid (A4-protein) obtained from Alzheimer's Disease.

The great radicular artery of Adamkiewicz in man. Demonstration of a possibility to predict its functional territory.

A women in her early sixties suffered from a malignant intramedullary astrocytoma. It had caused a nearly complete neurological deficit extending from the 12th thoracic segment downward. Since progressive tumour growth caused excruciating pain it was decided to remove the tumorous non-functional part of the spinal cord.

Hereditary cerebral hemorrhage with amyloidosis in patients of Dutch origin is related to Alzheimer disease.

Hereditary cerebral hemorrhage with amyloidosis in Dutch patients is an autosomal dominant form of vascular amyloidosis restricted to the leptomeninges and cerebral cortex. Clinically the disease is characterized by cerebral hemorrhages leading to an early death. Immunohistochemical studies of five patients revealed that the vascular amyloid deposits reacted intensely with an antiserum raised against a synthetic peptide homologous to the Alzheimer disease-related beta-protein.

The localization of protruded lumbar discs in advancing age.

The Leiden case material of 6,565 protruded/herniated discs is analyzed with regard to age and localization. It appears that a positive correlation exists between these two parameters: in advancing age the protruded disc shows an apparent shift in localization to higher lumbar levels, the incidence of its localization at the L5-S1 level decreasing markedly at the same time.

The carpal tunnel syndrome. The role of a persistent median artery.

After the first description by Paget (1854) and later on by Mohr (1890) and Schultze (1892), more than 100 years have passed by before it was generally recognized that compression of the median nerve in the carpal tunnel could be related to the majority of cases with nocturnal acroparaesthesiae in the hand and fingers. A short survey is given on 914 surgically treated patients (1117 operations). In one of them a bilateral carpal tunnel syndrome is related to a large persistent median artery.

The future role of neurosurgery in medicine.

Illustrated by several examples from the past it is demonstrated how difficult it is to predict future developments in neurosurgery. Several determinants can be recognized as exerting a positive or a negative influence in this respect. Careful observation of unexpected events and consequent consideration in combination with a spiritual independency, are aspects which will contribute in a positive way.

Metastasis of an occult gastric carcinoma suggesting growth of a prolactinoma during bromocriptine therapy: a case report with a review of the literature.

The treatment of a slowly growing invasive prolactinoma with bromocriptine for 8 months resulted in a substantial decrease in plasma prolactin levels despite rapid suprasellar tumor expansion. On exploration, this uncommon observation could be attributed to hematogenous metastasis from an occult gastric adenocarcinoma to the pituitary tumor. Apart from infiltration of neighboring parts of the hypothalamus, autopsy revealed no other hematogenous metastases.

Cervical myelopathy in rheumatoid arthritis.

Results obtained in 43 Rheumatoid arthritis (RA) patients with cervical myelopathy are described; all patients showed several alarm signs together with neurological disturbances. Thirty-four cases were operable; nine patients were not operated upon for various reasons (refusal, and general condition). In the surgically treated patients, the changes were localized in the C1-C2 area (n = 20), in the area below C2 (n = 5), or in both (n = 9).

The arteries of the spinal cord. The history of a paradigmal shift.

A historical review of the arterialization of the spinal cord is given. Against the background of philosophical attitudes in various periods during the last two centuries, it is explained how a paradigm may exert a negative influence on scientific progress.