T cell lymphocytosis associated with polymyositis, myasthenia gravis and thymoma.

Peripheral T cell lymphocytosis is a rare finding in association with malignant thymomas. In the majority of previous cases, the tumours have behaved aggressively with symptoms arising from local invasion. We describe a patient with ocular myasthenia gravis who presented with a rapidly progressive polymyositis and neuropathy and who was subsequently found to have a thymic mass and a mild T cell lymphocytosis. The thymoma did not give rise to local symptoms and showed no evidence of progression over a 14-month period of follow-up. The possibility of an underlying thymic tumour should be considered in any patient with chronic T cell lymphocytosis if the circulating cells show mature morphology and there is no molecular evidence of monoclonality.

Neuropathic urinary retention in the absence of neurological signs.

We present two cases of painless urinary retention secondary to central intervertebral disc prolapse. In neither case were there signs or symptoms suggesting an underlying neurological insult. Both patients voided spontaneously following neurosurgical intervention. The classical features of acute cauda equina compression may be absent in patients with central lumbar disc protrusion. Painless urinary retention may be the only physical sign.

Magnetic resonance imaging in degenerative ataxic disorders.

MRI of the brain was performed in 53 patients with a variety of degenerative ataxias and related disorders and 96 control subjects. Atrophy of intracranial structures was not seen in patients with the pure type of hereditary spastic paraplegia, or in early cases of Friedreich's ataxia. In advanced Friedreich's ataxia there was atrophy of the vermis and medulla. The MRI features of early onset cerebellar ataxia with retained reflexes were variable, and suggest heterogeneity. In autosomal dominant cerebellar ataxias, most patients had cerebellar and brainstem atrophy, probably reflecting the pathological process of olivopontocerebellar atrophy; there was no clearly defined group with both clinical and imaging features of isolated cerebellar involvement. The MRI abnormalities in idiopathic late onset cerebellar ataxia were predominantly those of cerebellar and brainstem atrophy or pure cerebellar atrophy. The clinical and imaging features of brainstem abnormalities were discordant in several patients. Pure cerebellar atrophy was associated with slower progression of disability. Cerebral atrophy was common in the late onset ataxias. Cerebral white matter lesions, although usually few in number, were observed in significantly more patients than controls, particularly those aged over 50 years.

Focal weakness following herpes zoster.

Three patients presented with focal weakness of an arm which followed segmental herpes zoster affecting the same limb. Neurophysiological investigations suggest that the site of the lesion lay at the root, plexus, or peripheral nerve level. This reflects the various ways in which the virus may affect the peripheral nervous system.

Strachan's syndrome: variation on a theme.

We report two patients of Afro-Caribbean origin with an ataxic neuropathy combined with visual loss and deafness. In previous reports of a similar syndrome most patients have been malnourished and have had mucocutaneous lesions, features which were absent in our patients.

Guillain-Barré syndrome after herpes zoster infection: a report of 2 cases.

We report 2 patients with Guillain-Barré syndrome following infection with the varicella-zoster virus. Evidence from neurophysiological studies is provided and the literature is reviewed on the association between these conditions.

Hereditary neuralgic amyotrophy associated with a relapsing multifocal sensory neuropathy.

A family with neuralgic amyotrophy (idiopathic brachial plexus neuropathy) associated with a multifocal sensory neuropathy is described. Four members over two generations were affected by neuralgic amyotrophy, inherited as an apparent autosomal dominant trait; two also had a multifocal relapsing sensory neuropathy with the clinical features of Wartenberg's migrant neuropathy.

Prolonged neurogenic weakness in patients requiring mechanical ventilation for acute airflow limitation.

We describe three patients who required mechanical ventilation for severe acute exacerbations of obstructive airways disease. When treatment with sedatives and muscle relaxants was withdrawn, they exhibited profound generalized weakness and consequently required prolonged ventilation despite resolution of the airway obstruction. Clinical features were variable, but none of the patients developed failure of other organs and infection was confined to the lungs. All had electrophysiologic evidence of a predominantly motor axonal syndrome. One patient in whom sensory action potentials were abnormal may represent an unusually severe case of critical illness neuropathy occurring in the absence of systemic sepsis and multiple organ failure. In the other two cases, this diagnosis is made less likely by the complete absence of sensory involvement and in these patients the lesion appeared to be either in the most distal portion of the motor neuron or at the neuromuscular junction. In all three patients, resolution was slow but eventually complete. The etiology of the condition is not clear, but it seems to be distinct from the acute myopathy previously described in asthmatics who had received mechanical ventilation. It is important to recognize this phenomenon to avoid erroneous conclusions about the likelihood of the patient recovering from ventilator dependence. A prolonged weaning period is to be expected in such cases.

Biochemical changes within a multiple sclerosis plaque in vivo.

Magnetic resonance spectroscopy (MRS) was performed on a superficial part of the brain containing a large multiple sclerosis (MS) lesion. Reduced levels of phosphocreatine (PCr) relative to adenosine triphosphate (ATP) were found suggesting an abnormality in energy metabolism, with an increase in the phosphodiester (PDE) peak. A follow up study 16 months later revealed reduction in size of the lesion on MRI and normal intracellular biochemistry by MRS. Four MS patients without visible superficial cerebral lesions showed no significant changes in phosphorus metabolism.

Involvement of the central nervous system in chronic inflammatory demyelinating polyneuropathy: a clinical, electrophysiological and magnetic resonance imaging study.

In a consecutive series of 30 patients with chronic inflammatory demyelinating polyneuropathy (CIDP) minor clinical evidence of CNS involvement was found in five. Cranial magnetic resonance imaging (MRI) was performed in 28 and revealed abnormalities consistent with demyelination in nine patients aged less than 50 years and abnormalities in five aged 50 years or over. Measurements of central motor conduction time (CMCT) were obtained in 18 and showed unilateral or bilateral abnormalities in six. It is concluded that subclinical evidence of central nervous system (CNS) involvement is common, at least in patients with CIDP in the United Kingdom, but that clinically evident signs of CNS disease are infrequent. The association of a multiple sclerosis-like syndrome with CIDP is rare.

Hemiparetic multiple sclerosis.

Eight patients are described who presented with hemiparesis which involved the face in seven. Six of the eight subsequently developed clinically definite multiple sclerosis and in the remaining two patients multiple sclerosis was the likely diagnosis. Magnetic resonance imaging gave useful information about the site of the lesions responsible for the presenting syndrome and provided additional information in support of a diagnosis of multiple sclerosis.

Magnetic resonance imaging of inflammatory and demyelinating white-matter diseases of childhood.

Magnetic resonance imaging (MRI) was performed on 36 children and two adults (with clinical presentation during childhood) with white-matter disease of the central nervous system. Abnormalities were readily demonstrated in patients with multiple sclerosis, acute disseminated encephalomyelitis, leucodystrophies and subacute sclerosing panencephalitis: MRI demonstrated the extent and distribution of abnormalities more clearly than computed tomography for all these disorders. The abnormalities tended to be asymmetrical and multifocal in multiple sclerosis and acute disseminated encephalomyelitis, and more confluent and symmetrical in the leucodystrophies. Children with clinically isolated optic neuritis had a significantly lower frequency of MRI brain-lesions than adults with the same disorder. MRI should be regarded as the radiological investigation of choice when white-matter disease is suspected in children.

The early risk of multiple sclerosis following isolated acute syndromes of the brainstem and spinal cord.

A combined clinical and magnetic resonance imaging follow-up study was undertaken to determine the risk of early progression to multiple sclerosis in patients who present with clinically isolated lesions of the brainstem or spinal cord. Progression to multiple sclerosis was seen in 13 patients (57%) who had a brainstem syndrome and in 14 patients (42%) who had a spinal cord syndrome after mean intervals of 15 and 16 months, respectively. The risk of progression was increased by the presence of oligoclonal bands in the cerebrospinal fluid of patients with either syndrome and by the presence of disseminated brain lesions, as detected by magnetic resonance imaging, in those with a spinal cord syndrome. Follow-up revealed evidence of multiphasic disease in 24 (69%) of 35 patients who had disseminated lesions on magnetic resonance imaging scans at presentation, suggesting that multiple sclerosis is the usual cause of such lesions.

Focal cranial nerve involvement in chronic inflammatory demyelinating polyneuropathy: clinical and MRI evidence of peripheral and central lesions.

Five cases of chronic inflammatory demyelinating polyneuropathy are described in which cranial nerve involvement accompanied a more generalized neuropathy. Clinical, electrophysiological, radiological and nerve biopsy findings are presented. Cranial nerve lesions in this form of polyneuropathy may be related to lesions of the peripheral nerves or of the central nervous system, when they may be accompanied by MRI evidence of more widespread CNS demyelinating lesions. In cases of early onset, the occurrence of focal cranial nerve lesions may serve to distinguish chronic inflammatory from inherited demyelinating polyneuropathies.

The early risk of multiple sclerosis after optic neuritis.

Serial brain MRI was performed in 53 patients with clinically isolated optic neuritis. Using clinical and imaging evidence for relapse, multiple sclerosis developed within a mean of 12 months in 19 of 34 cases (56%) with brain lesions at presentation, and in only 3 of 19 cases (16%) without (Relative Risk = 6.8, p less than 0.005).

Giant axonal neuropathy: observations on a further patient.

A further child with giant axonal neuropathy (GAN), abnormally curly hair and consanguineous parents is described. Of the 19 patients with GAN so far reported in the literature, six, including the present patient, have resulted from consanguineous marriages. This makes autosomal recessive inheritance of GAN highly probable. Our patient also exhibited cerebellar ataxia and signs of pyramidal tract damage; magnetic resonance brain imaging demonstrated abnormalities within the cerebellar and cerebral white matter. Myelinated nerve fibre density in the sural nerve was reduced to 6790/mm2 at age 8 years and had fallen to 3812/mm2 16 months later, indicating that progressive axonal loss occurs in GAN.

Magnetic resonance imaging in central nervous system sarcoidosis.

We performed brain MRIs on 21 patients with CNS sarcoidosis. Brain CTs were performed in 18 of these. Parenchymal lesions were seen in 17 of 21 with MRI, compared with 9 of 18 with CT. MRI detected a greater number of parenchymal lesions in cases where both CT and MRI were positive, and some lesions appeared more extensive with MRI than with CT. The most common MRI pattern was one of periventricular and multifocal white matter lesions (14 cases). Such a pattern is not specific, and other recognized causes for it were identified in four cases. It is likely, however, that sarcoid tissue causes this pattern in some cases, and confirmation was obtained from cerebral biopsy in one. In six patients, the white matter changes were indistinguishable from those seen in multiple sclerosis. Contrast-enhanced CT in two patients showed diffuse meningeal involvement not seen with MRI. MRI is the investigation of choice in detecting parenchymal changes in the brain of patients with CNS sarcoidosis and may prove useful in monitoring treatment in such cases.

Psychiatric and neurological manifestations in systemic lupus erythematosus.

The frequency and type of psychiatric disease were investigated in 40 patients suffering from systemic lupus erythematosus (SLE) and 27 control subjects with rheumatoid arthritis or inflammatory bowel disease. The psychiatric morbidity at the time of interview was the same in the two groups, but the patients with SLE had experienced more episodes of psychiatric illness in the past, and psychotic symptoms occurred only in this group. Half of the patients with SLE had previous or current evidence of neurological involvement; an association was found between neurological disease and psychotic symptoms in SLE, while anxiety and affective disturbances appeared to be closely related to environmental factors in both patients with SLE and controls. There was no correlation between psychiatric and neurological disease and clinical or laboratory indices of disease activity. Magnetic resonance imaging of the brain was performed in 15 patients with SLE. Abnormalities were more often present in those with neurological disease; no such correlation was found with psychiatric illness.