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A 79-year-old woman with persistent cortical blindness caused by bilateral temporo-occipital infarctions was followed for 8 months. She had no light or visual motion perception. Our patient's visual imagery was intact, which was demonstrated when drawing elementary shapes; however, her drawing of objects was poor. Optokinetic nystagmus could not be elicited, but the vestibulo-ocular reflex (VOR) seemed intact. Although she was able to suppress her VOR by 'fixating' her outstretched hand which moved in phase with her head and body, she showed saccadic tracking eye movements in an attempt to visually 'follow' the self-generated movements of her outstretched hand, while her body and head were stationary. Such saccadic tracking seemed to be different from the previously described smooth tracking of self-moved targets by patients with acquired blindness caused by anterior visual pathway dysfunction.

A 65-year-old man was suffering from recurrent manic psychosis accompanied by weight loss. He also had a history of pleural effusion, aspecific migratory non-deforming seronegative polyarthritis, sensorineural hearing loss and semicircular canal paresis. Whipple's disease (WD) had been diagnosed at the age of 63 years. On admission to hospital) he had weight loss, diarrhoea in combination with an organic brain syndrome, hemiparesis and ophthalmoplegia, including internuclear ophthalmoplegia (INO). A clinical diagnosis of central nervous system (CNS) WD was made. MRI revealed a thalamus lesion that halved in size during sulfamethoxazole-trimethoprim treatment. The organic brain syndrome and ophthalmoplegia diminished also, as did the cerebrospinal fluid (CSF) IgG level. A review of CNS WD is presented and implications for treatment are discussed.

Neurological, auditory, vestibular and ocular motor examinations were performed on 3 definite and 3 possible heterozygous carriers of a previously described X-linked multi-system disorder with early childhood onset, rapid progression and a fatal outcome (Arts et al., 1993). The symptoms, i.e., delayed motor development, ataxia, hearing loss and subnormal intelligence, were so evident in 2 of the possible carriers that they could be redesignated as probable carriers. Other symptoms in the definite and probable carriers were clubfeet, dysarthria, intention tremor and abnormal gait, while their signs included dysdiadochokinesia, ataxic paraplegia, abnormal muscle tendon reflexes and extensor plantar responses. All the symptomatic carriers developed moderate-to-severe sensorineural hearing loss with normal stapedial reflexes and brain stem auditory evoked potentials (BAEPs) in those in whom this could be evaluated. Speech discrimination was disproportionally poor unilaterally in one case from whom no BAEPs could be obtained because of her degree of hearing loss. Various combinations were found of high gain of the vestibulo-ocular reflex, spontaneous nystagmus and directional preponderance of vestibularly evoked nystagmus, slowing, hypometria or multi-stepping of saccades, saccadic intrusions of eye movements (macro square wave jerks, double saccadic pulses), impairment of smooth pursuit eye movements and optokinetic nystagmus, and failure of visual fixation suppression of vestibularly evoked nystagmus. Such findings indicate major involvement of the (vestibulo)cerebellum and the vermis. MRI in one carrier showed mild cerebellar atrophy.

Vestibular, oculomotor and respiratory tests were performed on 32 patients after whiplash injury caused by a rear-end car collision. Oculomotor functions were generally normal. The cervico-ocular reflex was usually absent or displayed the low gain typical of normal subjects. There was no nystagmic response to static neck torsion. The vestibulo-ocular reflex showed vestibular hyperreactivity (VH) in a significantly large number of cases (n = 17; 53%). The respiratory test results were also typical of the hyperventilation syndrome (HVS) in a significantly large number of cases (n = 12; 38%). The findings of VH and the HVS were not significantly correlated within the patient group. However, the combination of VH and the HVS occurred significantly more often (n = 7; 22%) than could be accounted for by combined false positivity. Most of the significant findings were due to high relative frequencies in the women: 11 out of the 17 women (65%) showed VH, 8 (47%) had the HVS and 5 (29%) showed a combination of VH and the HVS. The findings were not correlated with the patient's age or the time interval between the accident and the examination. VH might have been the result of plastic adaptation to limited head mobility secondary to neck pain. Behavioural and emotional distress might offer alternative explanations for both VH and the HVS.

Two males suffering from Pelizaeus-Merzbacher disease were examined, one at the age of 1 year 4 months and at the age of 7 years, and the other at the age of 7 years 8 months. The former had spontaneous vertical pendular nystagmus. He also showed horizontal "micronystagmus", present only at the age of 1 year, which might be similar to "voluntary nystagmus". Both males had jerky bilateral gaze-evoked nystagmus, defective smooth pursuit and optokinetic responses and a hyporeactive vestibulo-ocular reflex (VOR). All three obligate carriers exhibited typical VOR disinhibition in the two horizontal nystagmus directions, which may be a distinctive feature. This feature was also found in one of the 7 possible carriers examined and was not observed in the 3 non-affected males, who had normal oculomotor responses.

The results of clinical and electrophysiological investigations in a patient with a locked-in syndrome due to a pontine infarction, mainly on the right side, are presented. EEG showed only slight disturbances, while BAER and SSER revealed response alterations as could be expected from physical examination. BAER revealed altered central conduction after stimulation of either side. After median nerve stimulation on the right side SSER was slightly delayed, while no reproducible cortical response was seen after stimulation on the left side. Our patient survived and showed partial recovery.

A family is described with a progressive autosomal dominant vestibulocochlear dysfunction resulting in a Dandy syndrome, head movement dependent oscillopsia and hearing loss. The history was negative for other neurological or otological diseases (including infectious diseases) or use of neuro-ototoxic drugs, except for a high incidence of vascular disorders (hypertension, stroke, and heart infarction).

Three members of a single family with the symptom of "motion sickness" showed rebound nystagmus, saccadic pursuit eye movements, defective optokinetic slow phase velocity and lack of fixation suppression of vestibularly induced nystagmus. One of them showed vestibular hyperreactivity and a gradual build-up of the optokinetic response. In absence of other abnormalities, these findings can be localized to the vestibulocerebellum (flocculo-nodular lobe).