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A neuropathological and neuropsychological study of Turner's syndrome.

A neuropathological study was made in 2 women with Turner's syndrome. Neuropsychological investigation in one of them correlated with what has previously been found in Turner's syndrome as well as with the localization of the most pronounced neuropathological aberration which was of atherosclerotic nature, most pronounced in the right temporo-parietal area. These findings as well as the findings of acidophile hypoplasia of the pituitary gland are discussed.

Adult↗

Wallenberg's syndrome: neurotological classification.

OBJECTIVES: Visually induced eye movements were investigated in 26 patients with Wallenberg's syndrome. METHODS: Slow-phase optokinetic nystagmus (OKN) velocities, pursuit gains, and percentage fixation suppression (%FS) of caloric nystagmus were recorded by DC electrooculography (EOG), and stored directly onto FM magnetic tape. RESULTS: OKN velocities, pursuit gains, and %FS decreased toward the lesion side (group A), whereas OKN velocities and pursuit gains decreased toward the side contralateral to the lesion side. %FS decreased toward the lesion side (group B). MR images in group A showed that lesions were only in and near to the vestibular nuclei, and images in group B showed that lesions extended to the cerebellum. CONCLUSION: Visually guided eye movements allow classification of Wallenberg's syndrome into two types, one with brainstem lesions and one with both brainstem and cerebellar lesions. This demonstrates that assessing eye movements is helpful in supplementing MRI data.

Adult↗

The effects of reduced cerebrovascular circulation on the auditory brain stem response (ABR).

The effect of decreased anterior (carotid) bloodflow on the auditory brain stem response (ABR) was studied in a group of middle-aged males with angiographically confirmed decreased carotid circulation who were candidates for internal carotid end-arterectomy. Individual and mean latencies from the experimental group were compared to latencies of a control group matched for age, sex, and hearing loss. Mean absolute latency of wave V was significantly longer in the group with decreased anterior circulation when compared to the matched control group. These findings are similar to those from studies examining decreased posterior (vertebrobasilar) circulation. Both the matched control and experimental group were also compared to clinic norms and judged for abnormality. None of the control group ABRs were judged abnormal, after correction for hearing loss, while 29% of the ABRs were judged abnormal from the group with decreased anterior circulation. Postoperative testing was performed 1 week after surgery to determine any immediate effects of revascularization of the ABR. No changes in absolute or interpeak latencies were observed for either ear between pre- and postoperative testing. It is concluded that patients with confirmed decreased cerebrovascular circulation may show prolongation of absolute latency of wave V on ABR testing. Revascularization surgery in these patients appears to have no affect on their ABR within 1 week of surgery.

Adult↗

Basilar artery dissection.

Dissection of the basilar artery caused sudden coma and death in a 40-year-old man. Atypical clinical features were explained at necropsy. A ventral dissection of the artery within its outer layers resulted in destruction of the pontine tegmentum with sparing of the basis pontis. An unsuspected defect in the internal elastic lamina in the left internal carotid artery was also found suggesting a more generalised disorder of arterial walls. Basilar artery dissection should be considered in the diagnosis of coma in young people.

Adult↗

Binswanger's disease (Part II): Pathogenesis of subcortical arteriosclerotic encephalopathy and its relation to other dementing processes.

Subcortical arteriosclerotic encephalopathy (SAE) is a common though infrequently recognized dementia of the elderly. The unique vascular anatomy of the subcortical white matter and central brain stem probably predisposes those regions to chronic ischemia and incomplete infarction in the presence of various cardiovascular and hemodynamic insults. Recent studies have begun to define the risk factors for SAE, and others have shown it to be a condition frequently comorbid with the dementias of Alzheimer's disease, the multi-infarct state, and normal pressure hydrocephalus. Recent research into the etiologies of these disorders suggest certain pathogenetic links between them, strongly implying that they are not neatly distinct disease entities, as is commonly believed, and accounting for some of the overlap between these dementing illnesses seen clinically.

Alzheimer Disease↗

Management morbidity and mortality of poor-grade aneurysm patients.

Preliminary experience with the occasional good survival of patients in Hunt and Hess Grade IV or V with aneurysmal subarachnoid hemorrhage (SAH) led to a prospective management protocol employed during a 2 1/2-year period. The protocol utilized computerized tomography (CT) scanning to diagnose SAH and to obtain evidence for irreversible brain destruction, consisting of massive cerebral infarction with midline shift or dominant basal ganglia or brain-stem hematoma. These patients, along with those who exhibited poor or absent intracranial filling on CT or angiography, were excluded from active treatment and given supportive care only. All other patients had immediate ventriculostomy placement and, if intracranial pressure (ICP) was controllable (less than or equal to 30 cm H2O without an intracranial clot or less than or equal to 50 cm H2O in the presence of a clot), went on to have craniotomy for aneurysm clipping. Aggressive postoperative hypertensive, hypervolemic, hemodilutional therapy was subsequently employed. Of 54 patients with poor-grade aneurysms, ventriculostomy was placed in 47 (87.0%) and yielded high ICP's in the overwhelming majority, with the mean ICP being 40.2 cm H2O. Nineteen poor-grade aneurysm patients received no surgical treatment and survived a mean of 31.8 hours with 100% mortality. Thirty-five patients underwent placement of a ventriculostomy, craniotomy for aneurysm clipping and intracranial clot evacuation, and postoperative hypertensive, hypervolemic, hemodilutional therapy. The outcome at 3 months of the 35 patients who were selected for active treatment was good in 19 (54.3%), fair in four (11.4%), poor in four (11.4%), and death in eight (22.9%). It is concluded that poor-grade aneurysm patients usually present with intracranial hypertension, even those without an intracranial clot. Based on radiographic rather than neurological criteria, a portion of these patients can be selected for active and successful treatment. Increased ICP can be present without ventriculomegaly, and immediate ventriculostomy should be performed. As long as ICP is controllable, craniotomy and postoperative intensive care can effect a favorable outcome in a significant percentage of these patients.

Cerebral Hemorrhage↗

[An unconscious patients with ascending aortic aneurysm accompanied by aortic regurgitation successfully treated by emergency operation after cardiopulmonary resuscitation].

A-37-year-old woman in shock condition was transferred to our hospital after cardiopulmonary resuscitation for ventricular fibrillation. She was unconscious and suspected of suffering ischemic brain damage, with pathologic reflexes and weak brain stem reflexes. Brain CT scan showed cerebral edema without hemorrhage or infarction and an electroencephalograph revealed slow alpha-theta waves. Chest CT scan and echocardiogram showed ascending aortic aneurysm with sever aortic regurgitation. An emergent operation was performed for progression of heart failure. There were no distortion or dilatation of the sinus of Valsalva and annuloaortic ectasia and aortic valve leaflets were almost normal. We considered that the aortic valve dysfunction was cause by dilatation of the sinotubular junction. Ascending aortic and aortic valve replacement were carried out to shorten cardiopulmonary bypass time and to prevent the progression of brain damage. Mild hypothermia was employed as a neuroprotective procedure for three days after surgery. The patient's neurological symptoms, which were right hemiparesis, facial apraxia and motor aphasia, improved and she was discharged from the hospital on foot without any neurological complications on the 47th postoperative day and returned to work after two months.

Adult↗

Bilateral faciobrachial paresis as a consequence of symmetrical capsular infarcts.

A 32-year old man presented with a bilateral faciobrachial paresis, pyramidal signs in the upper limbs and dysarthria. Computer tomographic (CT-)scans showed bilateral cortical zones of contrast enhancement and strikingly symmetrical capsular hypodensities. Angiography revealed a stenosis of the left internal carotid artery and an occlusion of the right internal carotid artery. Essential thrombocythemia was diagnosed as the underlying disorder. Since there are no indications of pontine lesions, we assume that the signs and symptoms in this patient could mainly be attributed to the bilateral capsular lesions, that resemble lacunar infarcts.

Adult↗

[Persistent primitive first cervical intersegmental artery (proatlantal artery II) with occlusion of the basilar artery--a case report].

A rare case of persistent primitive first cervical intersegmental artery (proatlantal artery II) is reported. A 58-year-old man was admitted to our hospital with dysarthria and left hemiparesis. On admission he was stuporous with bilateral gaze palsy and left hemiparesis. CT scan on admission showed low density areas in the right cerebellar hemisphere and ventricular part of the pons. Right retrograde brachiography revealed occlusion of the basilar artery, aplasia of the right vertebral artery and an abnormal vessel connecting the right external carotid artery and the right vertebral artery. This anastomotic vessel was thought to be a persistent primitive first cervical intersegmental artery (Proatlantal artery II). Left carotid angiography revealed the left posterior cerebral artery was visualized through the posterior communicating artery, leading from the internal carotid artery. Left retrograde brachial angiography showed that the left vertebral artery terminated just distal from the branching of the left posterior inferior cerebellar artery. After admission the left hemiparesis deteriorated gradually and tracheotomy was done due to respiratory difficulties. The patient was then transferred to the rehabilitation center on his 34th day in hospital with neurological deficits.

Arterial Occlusive Diseases↗

Tandem bypass: occipital artery to posterior inferior cerebellar artery side-to-side anastomosis and occipital artery to anterior inferior cerebellar artery end-to-side anastomosis--a case report.

A unique example of posterior fossa revascularization is presented. A tandem bypass was performed by anastomosing the midoccipital artery to the posterior inferior cerebellar artery in a side-to-side fashion followed by anastomosis of the distal occipital artery to the anterior inferior cerebellar artery in an end-to-side fashion. The operation was designed to revascularize two separate vascular territories that were isolated in a patient thought to have an extremely compromised posterior circulation. The patient is doing well and is asymptomatic 3 years postoperatively.

Brain Stem↗

[Origin of olfactory and rhinosensory evoked cortical potentials in diseases of the central nervous system].

This is the first report to be published on olfactory evoked potentials in patients with well-defined lesions of the central nervous system and the trigeminal nerve. Absence of olfactory evoked potentials is seen in post-central and parietotemporal lesions. The first peak of the so-called olfactory evoked twin potential is absent in lesions of the basal nuclei and sectioning of the trigeminal or ophthalmic nerve, whereas there is no second peak in subcortico-frontal and cortico-temporal lesions. Tumours of the corpus callosum and sectioning of the maxillary and mandibular nerves do not disturb the olfactory evoked potentials. The anatomically different localisation and the functional synergism of the olfactory and trigeminal systems in the perception of odours and the processing of olfactory evoked potentials are pointed out.

Adolescent↗

[The locked-in syndrome. A case report].

Based on five of our own cases and the literature on hand the etiological, clinical, diagnostic and intensive therapeutic aspects of the syndrome are represented. Special emphasis is placed on the possible role of the oral contraceptives in triggering this syndrome.

Adult↗

Clinical detection of acute vestibulocerebellar disorders.

The acute onset of vertigo, nystagmus and postural instability, without brain-stem signs, is commonly attributed to a disorder of the labyrinth, the vestibular, sensory end organ. Identical symptoms can occur, however, with discrete infarctions or hemorrhages involving the central vestibulocerebellum. Whereas acute labyrinthine disorders are usually benign and self-limited, vascular injuries of the cerebellum may produce swelling, compression of the brain stem and acute hydrocephalus one to four days after the onset of symptoms. Therefore it is important to accurately distinguish between labyrinthine and vestibulocerebellar disorders with the neurologic examination. Acute labyrinthine disease causes unidirectional nystagmus with past-pointing and falling in the opposite direction of the nystagmus, environmental vertigo in the same direction and suppression of the nystagmus with visual fixation. Disorders of the vestibulocerebellum do not produce this consistent pattern of findings.

Cerebellar Diseases↗