Congophilic angiopathy of the brain: a clinical and pathological report on two siblings.

Clinical and histological accounts are given of a sister and brother, dying aged 61 and 56 years respectively after illnesses lasting 5-6 years marked by a progressive mental and physical disability. The family history suggested transmission by a dominant gene. The histological findings were of a very severe congophilic angiopathy confined to the brain, spinal cord and leptomeninges and giving rise to multiple haemorrhages and softenings. There were, in addition, abundant amyloid-containing "plaques" of various forms, found principally in the hippocampus and cerebellar cortex. The cases are compared with similar cases in the literature, and reasons given for regarding this condition as a separate entity rather than a variant of Alzheimer's disease.

Distribution of measles antigen and immunoglobulin-containing cells in the CNS in subacute sclerosing panencephalitis (SSPE) and atypical measles encephalitis.

The immunoperoxidase technique has been used to study the distribution of measles virus antigen and immunoglobulin (Ig)-containing cells within the CNS, in 5 cases of subacute sclerosing panencephalitis (SSPE) and 1 case of atypical measles encephalitis. Measles virus antigen was demonstrated within the brain in all cases, and in the spinal cord in 1 case of SSPE. Ig-containing cells were also demonstrated in all cases, the proportions of the different light and heavy chain types varying somewhat from case to case. In SSPE IgG constituted the major and IgA the principal minor heavy chain demonstrated. In all cases of SSPE there was significant excess of light-chain-containing over heavy-chain-containing cells. In the case of atypical measles encephalitis there was a paucity of Ig-containing cells and a relatively high proportion (39%) of these contained IgM. The case of atypical measles encephalitis differed from those of SSPE also in the presence of multinucleate giant cells, some of which contained measles virus antigen.

Laryngeal abductor paralysis in multiple system atrophy. A report on three necropsied cases, with observations on the laryngeal muscles and the nuclei ambigui.

Clinical and pathological findings are reported in three cases of Shy-Drager syndrome with laryngeal stridor severe enough to require tracheostomy. Indirect laryngoscopy in all three cases revealed a failure to abduct the vocal cords properly with a resulting narrowing of the glottic airway. Histological studies showed a marked atrophy of the posterior crico-arytenoid muscles and little atrophy of the other laryngeal muscles. The posterior crico-arytenoid muscles have the sole responsibility of abducting the vocal cords, so it is concluded that the patient's stridor was caused by the selective paralysis of these muscles. Histological studies of the posterior crico-arytenoid muscles were suggestive of denervation but despite this finding, no clear evidence of any motor cell loss in the nuclei ambigui was obtained. Other possible causes of the selective posterior crico-arytenoid atrophy are discussed.

Ponto-bulbar palsy with deafness (Brown-Vialetto-Van Laere syndrome).

The authors describe the clinical and electrophysiological features in 2 cases of ponto-bulbar palsy with deafness, and the clinical course and post-mortem findings in a sibling of one of these, who died in infancy, and who appears to have suffered from the same disease. The cases are compared with 17 previously published cases, and the disease is discussed in relation to other progressive neurological diseases of childhood.

Lateral horn cells in progressive autonomic failure.

Estimates of cell loss in the intermediolateral columns of the thoracic cord in cases of progressive autonomic failure were made by counting nerve cells in the lateral grey horns from 21 such cases, and comparing (a) with "normal" controls, (b) with 3 cases of uncomplicated Parkinson's disease and (c) with 3 cases of multiple system atrophy without overt autonomic failure. It was found that all cases of autonomic failure had suffered severe cell loss; on average three quarters of their lateral horn cells were lost. The cases of uncomplicated Parkinson's disease did not differ from the controls; but the cases of multiple system atrophy without autonomic disturbances had lost about half their lateral horn cells. These findings are tabulated, and discussed.

Multiple system atrophy with autonomic failure: clinical, histological and neurochemical observations on four cases.

Four cases of progressive autonomic failure are described, in all of which there were additional non-autonomic neurological abnormalities, including pyramidal, extra-pyramidal and cerebellar features. Histological examination revealed cell degeneration in the substantia nigra, putamen and intermediolateral columns of the spinal cord as a common pathological finding. In addition, 3 cases showed loss of Purkinje cells in the cerebellum and degeneration of pontine nuclei and inferior olivary nuclei. In one case there was cell loss from the locus coeruleus, caudate nucleus, vestibular nuclei and dorsal vagal nuclei. These were, therefore, cases of multiple system atrophy. Neurochemically, a common feature was a profound depletion in dopamine and noradrenaline from brain regions which are normally rich in these catecholamines. Central cholinergic systems appeared to be involved also, but to a variable degree.

Brain lesions in Friedreich's ataxia.

An estimate is given of the frequency of pathological changes (assessed by cell populations and from myelin staining) in the brains of 15 cases of Friedreich's ataxia. Mention is made of the special problem of distinguishing primary degenerative lesions from those due to circulatory disturbances arising from the patients cardiac disease.

Clinical and anatomic findings in a case of auditory agnosia.

A case is reported of severe agnosia for verbal and nonverbal sounds in a setting of well-preserved intelligence and transient dysphasic symptoms. The lesions responsible for this disorder were bilateral cerebral infarcts, one of which had virtually destroyed the areas of Wernicke and Broca. The relation between lesions and disabilities is discussed and comparisons made with other published cases.

The cervical cord in multiple sclerosis.

The spinal cords were examined in eighteen cases of multiple sclerosis, with special attention to the cervical enlargement. It was found that (1) lesions in the cervical cord are about twice as common as at lower levels, (2) in this region there is a striking preponderance of fan-shaped lesions in the lateral columns. It is argued that both these findings are explicable on the theory that mechanical stresses play a part in determining the site of lesions; that such stresses are commonly transmitted to the cord via the denticulate ligaments during flexion of the spine; and that many of the lesions are attributable to vascular leakages due to tension in the denticulate ligaments. It is concluded that in patients with multiple sclerosis neck flexion is dangerous--especially in cases where Lhermitte's sign has occurred.

Measles inclusion-body encephalitis in a child with treated acute lymphoblastic leukaemia.

A child with acute lymphoblastic leukaemia, being treated in the UKALL II Trial, had while in remission an attack of measles and made a normal recovery. Four months later she developed an acute encephalopathy and died within two weeks. The brain showed mild inflammatory features and widespread inclusion bodies in neurones and glial cells. Immunofluorescence proved an infection with measles virus. Similar cases have been called SSPE; reasons are given for preferring the term "measles inclusion-body encephalitis".

Malignant spread of haemangioblastoma: report on two cases.

Two cases are described in which, after successful removal of a cerebellar haemangioblastoma followed by several years of freedom from symptoms, there developed a progressive spinal cord compression, leading to death. At necropsy the spinal cords in both cases and the brainstem in one case, were irregularly plastered with haemangioblastoma. Although there was no doubt that malignant spread had occurred from one or more primary tumours, the histology of the tumour tissue was in no way different from that of conventional haemangioblastoma.

Late residua of acute idiopathic polyneuritis.

An account is given of four patients with acute idiopathic polyneuritis, leading within a few days to almost total paralysis. Two of these (cases 3 and 4) began to recover voluntary movement in the limbs in a month or less, and showed complete clinical recovery in three and 10 months respectively. The other two (cases 1 and 2) began to recover proximal limb movements after three months, reached a plateau of recovery in about two years, and never recovered movements in the distal parts of the limbs, which underwent muscular atrophy. Patient 2 died 14 years after the acute illness and was examined post mortem. The difference in recovery is explained by supposing that in patients 3 and 4 the lesions consisted predominantly of segmental demyelination, whereas in patients 1 and 2 there was extensive axon destruction at a proximal level. Recovery in the latter depended upon nerve regeneration, which restored the power of the proximal muscles, but was too slow for effective reinnervation of distal muscles. This explanation is supported by post mortem findings in patient 2. A further observation in patient 2 was of degeneration of the posterior white columns of the spinal cord, which was not due to loss of posterior root fibres. It is believed that in such cases a prognosis as to ultimate recovery of muscle power can be made about a month after the acute phase, according to whether movement has begun to return in the distal parts of the limbs. If recovery does not occur within two years it will not occur at all.

Neurogenic muscle atrophy in myasthenia gravis.

Two cases of severe myasthenia gravis are described, with histological findings. Both cases showed severe neurogenic atrophy of the muscles of the tongue, with fatty pseudohypertrophy and a remarkable proliferation of terminal nerve fibres. Similar, but less severe, changes were present in other bulbar muscles. The findings are in keeping with the view that functional interruption at the neuromuscular junctions results first in failure to transmit the contractile impulse, and at a later stage in denervation atrophy. Terminal proliferation of axons is regarded as an attempt, on the part of motor fibres, to compensate for the breakdown of normal neuromuscular interaction.

The central nervous system in motor neurone disease.

Forty-five necropsied cases with primary degeneration of lower motor neurones are described and discussed. Of these, 36 are considered to be `typical' cases of motor neurone disease, eight of which showed no upper motor neurone lesions. The relation of the nine `atypical' cases to the remainder is discussed. It is concluded that motor neurone disease constitutes an ill-defined band in a broad spectrum of multiple system atrophies. The authors have found no evidence suggesting a causal relation between motor neurone disease and either vascular or malignant diseases. They point out suggestive analogies with various subacute encephalomyelopathies in man and other animals.