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Hunter disease/mucopolysaccharidosis type II/iduronate sulfatase deficiency
Published in William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop, Atlas of Inherited Metabolic Diseases, 2020
The voice is hoarse. Diarrhea may be a chronic problem; it may result from infiltration of the autonomic innervation of the intestine [23]. Retinitis pigmentosa may occur in this condition and retinal degeneration may cause blindness. Glaucoma may be a problem. Papilledema may be seen [24]; this is probably a consequence of pachymeningeal thickening, which may also lead to neurologic defects including quadriplegia from pressure on the cord [25]. It may also result in hydrocephalus [26]. Cerebral atrophy, which may also lead to ventricular enlargement, is seen regularly on computed tomography (CT) scan or magnetic resonance imaging (MRI) in severe Hunter disease [27–29], and there may be defective reabsorption of cerebrospinal fluid. Intracranial pressure may be increased.
Basic observations
Published in Barbara Smith, Linda Field, Nursing Care, 2019
Intracranial pressure is the pressure within the skull bone.The diaphragm forms a large dome-shaped partition that separates the thoracic cavity from the abdominal cavity. It consists partly of muscle and partly of membrane. It is a very important muscle of respiration, contracting during inspiration to enlarge the thoracic cavity and relaxing during expiration.
Hepatic failure
Published in Michael JG Farthing, Anne B Ballinger, Drug Therapy for Gastrointestinal and Liver Diseases, 2019
In all encephalopathic ALF patients monitoring to anticipate the development of raised intracranial pressure is necessary before irreversible neurological injury has developed. Several monitoring modalities have been advocated and of these, direct measurement of intracranial pressure is most commonly practised.
Clinical aspects of malarial retinopathy: a critical review
Published in Pathogens and Global Health, 2023
Ketan Raymond Nair Brodeur, Anderson Herculano, Karen Oliveira
Some of the complications of severe malaria include severe anemia, metabolic acidosis, respiratory distress, and cerebral malaria, which is considered the most extreme form of severe malaria. While cerebral malaria (CM) is a rare condition affecting less than 1% of malaria cases, it is one of the deadliest complications with a mortality rate of 15–20% of those treated with ACT and an almost 100% fatality rate without treatment. Children under 5 years of age are some of the most vulnerable to cerebral malaria. CM is an incompletely understood complication and the exact pathogenic mechanisms are still unknown. The current understanding of the pathology hypothesizes that infected red blood cells (iRBC) adhere to endothelium cells in the brain and retina where they remain sequestered in the microvasculature. The iRBC are attached to the endothelium mediated by proteins such as Plasmodium falciparum erythrocyte membrane protein-1 [4]. This can lead to complications including obstruction of blood vessels, reduced perfusion, hypoxia and ischemia, inflammation due to increased expression of inflammatory cytokines, among other immune system agents, and increased brain volume and intracranial pressure [5–7]. Findings further suggest that such an increase in intracranial pressure is associated with brain swelling and are strong predictors of death [5].
Dural venous sinus stenting in patients with idiopathic intracranial hypertension: report of outcomes from a single-center prospective database and literature review
Published in Expert Review of Ophthalmology, 2022
Matthew J Kole, Juan Carlos Martinez-Gutierrez, Francisio Sanchez, Rosa Tang, Peng Roc Chen
A broad differential should be considered when patients present with elevated intracranial pressure, as several conditions can mimic the signs and symptoms of IIH. Intracranial mass lesions can cause elevated intracranial pressure, but these are easily diagnosed with CT or MRI. Venous sinus thrombosis can mimic IIH, with patients presenting with vision loss, headache, and papilledema [52]; the diagnosis is more common in young females, particularly those taking hormone-based oral contraception, or patients with clotting disorders [53]. The clinical presentation may reflect a more sudden course of onset. A patient with a dural arteriovenous fistula (dAVF), an abnormal connection between arteries within the dura and cortical veins and/or venous sinuses, may also present with similar findings as IIH, including pulsatile tinnitus with signs and symptoms of elevated intracranial pressure [54,55]. Noninvasive vascular imaging findings for dAVF are most often nonspecific, and a cerebral angiogram is required to make the diagnosis. Migraines may also mimic the headache and vision components of IIH, but papilledema and elevated opening pressure are absent. Vitamin A overdoses can also mimic IIH [56].
Intermediate surgical outcome in patients suffering poor-grade aneurysmal subarachnoid hemorrhage. A single center experience
Published in International Journal of Neuroscience, 2021
Anastasia Tasiou, Alexandros G. Brotis, Thanasis Paschalis, Christos Tzerefos, Eftychia Z. Kapsalaki, Theofanis Giannis, Alkiviadis Tzannis, Kostas N. Fountas
Seventeen cases (74%) underwent surgical clipping within 24 h from the initial symptom onset (Table 1). Nine cases (39%) had an intraparenchymal and/or a subdural hematoma associated with the ruptured aneurysm. An external ventricular drain (EVD) was inserted in 21 patients (91.3%). Elevated intracranial pressure was observed in 21 of our patients (91.3%). Seven patients (30.4%) developed persistent, medically intractable intracranial hypertension (ICP ≥30 mmHg), despite adequate CSF drainage through a previously inserted EVD. A standard pterional craniotomy (PT) was performed in ten (43.5%) patients, while thirteen (56.5%) underwent an extensive (more than 12 cm in its largest diameter), unilateral decompressive craniectomy (DC). Two patients (9%) developed post-hemorrhagic hydrocephalus, and a ventriculo-peritoneal shunt insertion was required. Five patients (21.7%) developed massive brain edema and finally succumbed to this.