Subacute progressive sensory ataxic neuronopathy after Rickettsia conorii infection.

A case is presented in which sensory ataxic neuronopathy developed after serologically proven infection with Rickettsia conorii and continued to be progressive after appropriate antibiotic treatment. Electrophysiological studies showed decreasing sensory nerve conduction velocities ending with the absence of sensory nerve action potentials as well as peripheral and cortical somatosensory evoked potentials. Histological studies revealed a profound loss of myelinated fibres due to primarily axonal degeneration.

Aware or non-aware. On the significance of awareness for the localization of the lesion responsible for homonymous hemianopia.

Some of the patients with homonymous hemianopia are unaware of any visual defect, whereas others perceive this defect more of less consciously. Analysis of the CT scans of 41 cases showed that patients who are partially or fully aware of the hemianopia have purely occipital lesions. The patients who were not aware of the visual defect were found to have larger and more anteriorly situated lesions.

The Brown-Séquard syndrome. True or false?

In recent years the name of Brown-Séquard has been assigned to syndromes caused by spinal cord damage differing widely in extension. On the basis of a review of more than 600 published cases, we propose that Brown-Séquard-plus syndromes be distinguished from the pure Brown-Séquard syndrome. This distinction is shown to be justified on clinical, anatomic, and historical grounds.

Transient global amnesia after coronary angiography.

A 69-year-old woman suffered transient global amnesia as a complication following coronary angiography. Three reports have been described earlier. The etiology of this condition is discussed.

The influence of physostigmine on visual-vestibular interaction in hereditary ataxias.

Visual suppression of caloric nystagmus was studied in five patients with hereditary ataxia before and after administration of physostigmine. All patients had an initial abnormal ocular fixation index that improved after physostigmine was given. The data indicate that there is a partly reversible disturbance of visual-vestibular interaction in patients with hereditary ataxia, caused by an impairment of a central cholinergic mechanism.

A rare cause of lumbosacral plexus neuropathy.

This is the second report of a case in which a uterine myoma, one of the most common benign tumours of women, was the cause of a lumbosacral plexus neuropathy. The possibility of uterine myoma should be considered in the differential diagnosis of neuropathy of the lumbosacral plexus in women.

Aphasia with a left frontal interhemispheric hematoma.

We studied a patient with transcortical motor aphasia resulting from a traumatic interhemispheric left frontal hematoma. The aphasia was caused by compression exerted by the hematoma on the left supplementary motor area, which is known to have a function in speech. This cause of a transcortical motor aphasia has not been described earlier.

Persistence of the blink reflex to sudden illumination in a comatose patient. A clinical and pathologic study.

A persistent blink reflex to light (BRL) was observed in a patient who became comatose after a cardiac arrest. Postmortem examination revealed severe hypoxic ganglion cell necrosis of the cerebral and cerebellar cortex, the basal ganglia, the hypothalamus, nuclei in the brain stem, and the superior colliculi. The pretectum was unaffected.

Thyroid crisis presenting as coma.

Coma is a rare complication of thyrotoxicosis. A patient with coma due to a thyroid crisis is reported. The EEG showed extremely slow and low voltage activity, which gradually returned to normal.

[Neurology and photographic illustration in the medical book].

The mutual influence between G. B. A.

Hereditary Pick's disease: second re-examination of the large family and discussion of other hereditary cases, with particular reference to electroencephalography, a computerized tomography.

A large family with Pick's disease was re-examined after twenty years. Five new cases were found, one in the fourth and four in the fifth generation. This family now includes 25 patients with the clinical diagnosis Pick's disease, proven by autopsy in 14, and 7 patients in whom the same diagnosis was considered likely, over six generations.