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Copyright of Journal of Neurology is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Journal of Neurology is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Archiv für Psychiatrie und Nervenkrankheiten is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

We present a patient who experienced sudden onset of orbital headache, visual loss and bitemporal visual field defect. MRI of the optic chiasm suggested a diagnosis of haemorrhage and hence a vascular malformation. Pterional craniotomy revealed an intrachiasmatic haematoma with a cavernous angioma. The malformation was totally excised and vision improved after surgery. The syndrome of chiasmal apoplexy is discussed. [ABSTRACT FROM AUTHOR]

Three young women (aged 18 years, 19 years and 19 years) who developed progressive neuropsychic and neurologic disturbances with hearing loss and multifocal retinal artery branch occlusions are reported. This retinocochleocerebral syndrome has been reported previously only in 12 young North American women. Its pathogenesis is unknown, but an atypical viral infection of the vessel walls has been suggested. Abnormalities of T lymphocytes subsets in blood in one of the patients suggested an immunological dysfunction, but all other tests, including immunological reactions on brain and skin biopsies, were negative or non-specific. Steroids and immunosuppressive agents have been advocated on an empirical basis, but the second patient showed a substantial recovery without any therapy and the third gradually deteriorated despite azathioprine, cyclophosphamide, prednisone and plasma exchanges. This retinocochleocerebral syndrome probably corresponds to an arteriolopathy of unknown nature. [ABSTRACT FROM AUTHOR]

A disinhibition syndrome affecting speech (with logorrhoea, delirium, jokes, laughs, inappropriate comments, extraordinary confabulations), was the main manifestation of a right-sided thalamic infarct involving the dorsomedian nucleus, intralaminar nuclei and medial part of the ventral lateral nucleus. Resolution of conflicting tasks was severely impaired, suggesting frontal lobe dysfunction. These abnormalities correlated with the finding on SPECT of a marked hypoperfusion in the overlying hemisphere predominating in the frontal region. We suggest that this behavioural syndrome was produced by disconnecting the dorsomedian nucleus from the frontal lobe and limbic system. [ABSTRACT FROM AUTHOR]

A macular-sparing superior altitudinal hemianopia with no visuo-psychic disturbance, except impaired visual learning, was associated with bilateral ischaemic necrosis of the lingual gyrus and only partial involvement of the fusiform gyrus on the left side. It is suggested that bilateral destruction of the lingual gyrus alone is not sufficient to affect complex visual processing. The fusiform gyrus probably has a critical role in colour integration, visuo-spatial processing, facial recognition and corresponding visual imagery. Involvement of the occipitotemporal projection system deep to the lingual gyri probably explained visual memory dysfunction, by a visuo-limbic disconnection. Impaired verbal memory may have been due to posterior involvement of the parahippocampal gyrus and underlying white matter, which may have disconnected the intact speech areas from the left medial temporal structures. [ABSTRACT FROM AUTHOR]

In a patient with a unilateral embolic infarct in the left posterior thalamo-subthalamic paramedian artery territory, neuropathological studies showed involvement of the intralaminar, dorsomedial, and internal part of the ventral posterior nuclei of the thalamus, of the rostral part of the mesencephalic reticular formation, and of the posterior commissure. The patient showed upgaze palsy for voluntary saccades, smooth pursuit and vestibulo-ocular movements, sustained downgaze, right-sided motor hemineglect and facio-brachial hypaesthesia, motor transcortical aphasia and anterograde amnesia. This case confirms that unilateral destruction of the posterior commissure, rostral interstitial nucleus of the MLF and interstitial nucleus of Cajal produces a non-dissociated upgaze palsy. Involvement of the nucleus of Cajal probably produced the sustained downward deviation of the eye, by causing predominance of downward vestibulo-ocular inputs. This case also shows that thalamic aphasia and anterograde amnesia may be related to a paramedian lesion of the thalamus, with special reference to involvement of the dorsomedial nucleus, in the absence of lesion of the pulvinar and mamillo-thalamic tract and of conspicuous involvement of the ventral lateral nucleus. Selective hemineglect for motor tasks may occur in infarction of the dominant thalamus, involving the intralaminar nuclei. [ABSTRACT FROM AUTHOR]

Five patients with "locked-in" syndrome and dysconjugate palsy of horizontal gaze were studied. In all cases internuclear ophthalmoplegia due to dysfunction or destruction of the median longitudinal fasciculus was combined with an ipsilateral gaze palsy, producing the "one-and-a-half" syndrome. Clinical and electro-oculographic examination suggested involvement of the paramedian pontine reticular formation when all ipsilateral saccades were abolished, when exotropia of the contralateral eye was present, and when vestibular stimulation showed full conjugate deviation to the damaged side. Involvement of the abducens nucleus was suggested when the palsy of ipsilateral gaze was not dissociated on vestibular stimulation. In three cases these clinical deductions were confirmed by the pathological study, which showed a corresponding destruction of the median longitudinal fasciculus, paramedian pontine reticular formation and abducens nucleus. In one case the one-and-a-half syndrome evolved into a total horizontal gaze palsy, which corresponded to involvement of the abducens nucleus contralateral to the initially destroyed paramedian pontine reticular formation. Vertical oculocephalic response disappeared, because of destruction of the median longitudinal fasciculus on both sides (bilateral internuclear ophthalmoplegia). Patients with the locked-in syndrome provide a unique situation in which complex pontine oculomotor disturbances may be studied, because consciousness is preserved. In these patients, dissociated and dysconjugate oculomotor palsy may have been underestimated. [ABSTRACT FROM AUTHOR]

Three cases of neuropathy with monoclonal gammopathy and amyloid deposits in peripheral nerves are described. They appeared to present a benign gammopathy because of the duration of the neuropathy in the absence of any clinical or biological sign of myeloma or macroglobulinaemia. The pathological abnormality found in the sural nerves of the three patients was characterised by a marked loss of myelinated and unmyelinated nerve fibres because of active axonal degeneration with Wallerian degeneration. The most striking feature in all three cases was the finding of deposits identified as an accumulation of microfibrils. [ABSTRACT FROM AUTHOR]