Cases reported "Quadriplegia"

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1/10. Effects of stereotactic lesions of the pulvinar and lateralis posterior nucleus on intractable pain and dyskinetic syndromes of man.

    In a series of 18 patients suffering from intractable pain or different types of dyskinetic syndromes, 28 stereotactic lesions of the pulvinar, associated with six lesions of the laterlis posterior nucleus, have been performed. The evaluation of long-term results in intractable pain reduces the therapeutic benefit of the stereotactic pulvinolysis. Concerning dyskinetic syndromes, the pulvinar does not seem to play an important role in spasticity, while its role in other dyskinetic syndromes can be questionable.
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2/10. Mini-infarct encephalopathy associated with uncommon microvessel convolute formation presenting with presenile dementia.

    A female patient started to suffer from transient ischemic attacks when she was 47 years of age, followed by increasing predominantly left-side spastic tetraparesis, generalized seizures and progressive dementia over a period of 11 years. She died when she was 58 years of age. On gross examination the brain showed enlarged ventricles and arteriosclerotic changes of large extracerebral vessels of the circulus arteriosus. Microscopic examination of the atrophic brain showed innumerable incomplete microinfarcts in the white and gray matter throughout all parts of the brain. In the white matter these lesions were characterized by small foci of demyelination and loss of oligodendrocytes while occasionally some scavenger cells were seen. axons seemed to be unaffected or displayed irregular axonal regeneratory growth. Any inflammatory reaction failed. In the cerebral cortex and subcortical nuclei the lesions showed loss of neurons and decrease in synaptophysin expression. Intracerebral arteries showed fibrosis or fibrohyalinosis of the entire intracerebral small-vessel network. In addition, numerous uncommon clusters of angioma-like telangiectatic vessels were observed. Medium-sized ischemic infarcts were found in the right putamen and adjacent internal capsule region, left-side dorsolateral brain stem and cerebellar hemisphere as well as a left-side pyramidal tract degeneration. Contralateral pseudohypertrophy of the inferior olivary nucleus was seen. The clinical and the neuropathologic observations made in this patient are compatible with small vessel disease characterized by a multicentric special and not yet described type of incomplete mini-infarcts in cerebral cortex and white matter accompanied by some larger ischemic infarcts of the common type in brain stem and cerebellum.
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3/10. A blessing in disguise: resolution of tardive dyskinesia with development of cervical myelitis.

    Tardive dyskinesia (TD), which is frequently seen in patients treated with dopamine receptor blocking agents, is difficult to manage. We report on a young Chinese man with bipolar disorder who developed TD after haloperidol treatment, involving the trunk, limbs, and orofacial area. TD persisted despite switching to atypical antipsychotic agents and treatment with valproate, benzodiazepines, and tetrabenazine. Resolution only occurred years later when he developed quadriplegia arising from infective myelitis of the cervical cord (C4-5). He had concomitant vertebral osteomyelitis, which was successfully treated with intravenous antibiotics. With intensive rehabilitation, he recovered the use of his limbs, but had no recurrence of TD. We attribute the resolution of orofacial dyskinesias with a cervical lesion to the interconnections between the orofacial area and cervical spine via the trigeminal nucleus (which has fibers descending as far caudally as C6), as well as to resetting of cortical maps.
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4/10. perfusion and diffusion tensor imaging in a patient with locked-in syndrome after neurosurgical vascular bypass and endovascular embolization of a basilar artery aneurysm: case report.

    OBJECTIVE AND IMPORTANCE: Locked-in syndrome is a state of preserved consciousness in the setting of quadriplegia, anarthria, and usually also includes lateral gaze palsy. It is most commonly associated with upper brainstem infarction variably sparing the third cranial nerve nucleus. There are likely many etiologies that contribute to this clinical syndrome. These are incompletely understood, and the syndrome remains a rare but devastating complication that can occur after neurosurgical and neurovascular interventions. Advanced magnetic resonance imaging techniques such as perfusion and diffusion tensor imaging may help to elucidate the mechanism behind locked-in syndrome. To the authors' knowledge, there are no reports in the literature of perfusion and diffusion tensor findings in patients with this syndrome. A postprocedural case of locked-in syndrome is described with abnormalities on perfusion and diffusion tensor imaging in the absence of any changes in conventional magnetic resonance imaging. CLINICAL PRESENTATION: A 57-year-old man who presented with acute onset headache, ataxia, and other nonspecific symptoms was found on imaging to have a giant fusiform basilar artery aneurysm. INTERVENTION: A saphenous vein graft bypass between the proximal right external carotid artery and P2 segment of the right posterior cerebral artery followed immediately by endovascular embolization of the aneurysm sac and distal left vertebral artery was performed. CONCLUSION: Postprocedural angiography demonstrated patency of the bypass graft, and diffusion weighted imaging showed no evidence for acute brainstem infarction. Nevertheless, despite technically successful procedures and the absence of abnormalities on conventional magnetic resonance imaging, the patient developed quadriplegia and anarthria and remained in a locked-in state until he expired. Abnormalities were, however, seen on both perfusion and diffusion tensor imaging, where hypoperfusion, increased mean diffusivity, and decreased fractional anisotropy were observed in the ventral brainstem. The findings suggested a disruption of pontine white matter tracts. Advanced imaging techniques may allow us to image important microstructural changes that were previously not discernable and assist in the evaluation of patients with complex neurological sequelae such as locked-in syndrome.
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keywords = nucleus
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5/10. Vertical gaze palsy and selective unilateral infarction of the rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF).

    We report a clinico-pathological correlation study in a patient with basilar artery thrombosis, who developed tetraplegia and combined up- and downgaze palsy involving voluntary saccades and visually-guided movements, but sparing the oculocephalic responses. At necropsy, apart from bilateral infarction in the basis pontis, there was a single unilateral infarct selectively destroying the rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF) on the right. The posterior commissure and its nucleus, the nucleus of Cajal, the nucleus of Darkschewitsch and the pontine tegmentum were spared. We suggest that the unilateral riMLF lesion may have disrupted bilateral upgaze excitatory and inhibitory inputs and unilateral downgaze excitatory inputs. The functional anatomy of inhibitory and excitatory vertical gaze circuitry, which remains speculative, may explain why a unilateral lesion of the upper midbrain tegmentum may be sufficient to generate an upgaze palsy or a combined up- and downgaze palsy, while an isolated downgaze palsy requires bilateral lesions.
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ranking = 1.6
keywords = nucleus
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6/10. Porencephaly and hydranencephaly: a neuropathological study of four autopsy cases.

    Four autopsy cases of porencephaly and hydranencephaly were evaluated clinico-pathologically. The patients showed profound mental retardation, convulsive seizures and spastic quadriplegia. The life spans were much shorter in the hydranencephalic cases. Pathologically, the cerebral cortex in the vicinity of the parenchymal defects often showed an abnormal cytoarchitecture, which appeared to be closely correlated with superficial glial proliferation. Several neurofibrillary tangles were found in the nucleus basalis of Meynert and the locus ceruleus in a 23-year-old porencephalic patient, and so-called grumose degeneration of the dentate nucleus in three patients, including a 2-month-old hydranencephalic boy. It is suggested that extensive cerebral defects may cause neuronal degeneration of the subcortical nuclei.
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keywords = nucleus
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7/10. Olivary hypertrophy in a case with palatal myoclonus: light- and electron-microscopic study.

    This is a report on the ultrastructural finding of the olivary hypertrophy in a case with palatal myoclonus. By light microscopy two types of neuronal changes were observed in the inferior olivary nucleus, i.e. the central chromatolysis and cytoplasmic vacuolation. Both types were also recognized by electron microscopy and the cytoplasmic vascuolation was identified as the vesiculated endoplasmic reticulum. In the reactive astrocytes, mitochondria were strikingly proliferated.
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ranking = 0.2
keywords = nucleus
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8/10. One-and-a-half syndrome in ischaemic locked-in state: a clinico-pathological study.

    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.
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ranking = 0.6
keywords = nucleus
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9/10. Dyspnoea during generalised spasms in tetraplegic patients.

    Dyspnoea during generalised spasms is reported in three tetraplegic patients. Intraoesophageal electromyographic evidence strongly suggests that the dyspnoea is a result of diaphragmatic spasms. Utilisation of this EMG technique in other tetraplegic patients may add to our knowledge of variations in the distribution of the phrenic nerve nucleus within the spinal cord.
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ranking = 0.2
keywords = nucleus
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10/10. Clinical, physiologic, and pathologic evidence for vagus dysfunction in a case of traumatic brain injury.

    A trauma victim with locked-in syndrome demonstrated severely decreased bowel sounds, intact response to suppository, and elevated, but unchanging pulse. Absent cardiac response to tracheal suctioning, high gastric residual volumes, and pulmonary edema in response to a urecholine challenge demonstrated dysfunction in the automatic system. Symptoms persisted for 2 1/2 years until death. At autopsy, asymmetric bilateral involvement of the dorsal motor nucleus of the vagus and of the nerve tract in the medulla were demonstrated. In contrast, a control subject with locked-in syndrome caused by a stroke did not demonstrate these phenomena. In trauma patients with delayed gastric emptying, measurement of the heart rate response to deep suctioning may lead to the diagnosis of this vagus dysfunction syndrome.
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keywords = nucleus
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