Acute Non-communicating Hydrocephalus Developed within 48 Hours in a Patient with Systemic Lupus Erythematosus

Hydrocephaly is a rare manifestation of systemic lupus erythematosus [SLE], and the pathogenesis is still unclear. Some studies suggest cerebral venous thrombosis, immune complex deposition within the arachnoid villi, or direct post-inflammatory lesions of the central nervous system [CNS] as possible causes, but these remain unproven. We report a case of acute non-communicating hydrocephalus secondary to stenosis of the aqueduct of Sylvius. The condition developed within a 48-hour period as the result of cerebrovascular accident in a 40-year-old man with previously diagnosed SLE. The pathophysiologic mechanism of hydrocephalus in SLE is subject to various arguments and remains a dilemma facing researchers. Case Report Adam et al.; INDJ, 8(3): 1-6, 2016; Article no.INDJ.31801 2


INTRODUCTION
Genetic and acquired etiologies of hydrocephalus have been identified, each with different pathogeneses and pathophysiologic mechanisms. SLE is a systemic autoimmune connective tissue disease with a variety of clinical manifestations that affect various internal organs, oral and conjunctival mucosa, cardiac and blood vessel endothelium, pericardial serosa and valves of the heart, joints, muscles, skin, lung pleura, liver, kidney glomeruli, and the central and peripheral nervous systems [1,2]. A review of the literature shows that during any time point in the course of SLE up to 50% of patients show neuropsychiatric involvement [3]. Hydrocephalous is a rare manifestation of SLE associated with central nervous system [CNS] involvement. In this case we report a patient with acute hydrocephalous developed within 48 hours as a result of an SLE-related cerebrovascular accident. We review the literature and attempt to prove the pathogenesis and pathophysiology of acute hydrocephaly associated with systemic lupus erythematosus. The pathogenesis and pathophysiologic mechanism of hydrocephalous associated with SLE are yet unproven [4], although several alternative mechanisms have been proposed in the literature. One hypothesis is that corticosteroids and other immune suppressive agents used in the treatment of SLE can lead to increased risk of opportunistic CNS infection, and these infections can lead to impairment of CSF and subsequent hydrocephalus [5]. Normal pressure hydrocephalus [NBH] in a 77-year-old patient with SLE has been reported, but no cause was found [6]. On the other hand, Krauss and associates showed a highly significant association between idiopathic NPH and arterial hypertension [7].

CASE PRESENTATION
Verrees hypothesizes that the development of hypertension beyond the limits of cerebral autoregulation leads to breakdown of the bloodbrain barrier in the cerebellum and development of posterior fossa edema secondary to focal transudation of protein and fluid. All of these studies show hypertension as an impotant contributing cause of NPH and obstructive hydrocephalus due to vascular encephalopathy [8]. In some lupus patients, hyperviscosity disrupt blood flow and might be involved in hydrocephalus [9]. Immune complex deposition can affect the brain parenchyma directly, or within the cerebrovascular system that can impair CSF flow into the arachnoid villi. Thromboembolic formation that blocks the small arteries, choroid plexus or cerebral venous system can be conceived as another pathophysiologic mechanism to explain the development of intracranial hypertension [4]. Kitching Fig. 3]. We could not have a control diffusion MRI for comparison, but the last control cranial CT was demonstrative to some extent. However, we were able to observe only the narrowing of the basal cisterns, partial narrowing of aqueduct cerebri and the hydrocephalus. It is worthy to mention that specific serological tests, radiological modalities such as PET CT, functional MRI, MRI angiography, CSF flow MRI and venography [to detect the pathogenesis of dural sinuses obstruction and aqueduct stenosis] and Digital Substraction Angiography [DSA] are of extreme important to provide informations and subsequently in evaluation of neuropsychiatric SLE patients. Unfortunately we couldn't obtain such investigation methods for our case to obtain evidents that strength our data and to prove our argument.

. Axial plan cranial CT approximately 48 hours after the previous cranial CT revealed noncommunicating hydrocephalus; also observe the narrowing of the basal cister
To discern the pathogenesis and pathophysiologic mechanisms of hydrocephalus in the case reported here, the most important question to be answered is how hydrocephalus developed within 48 hours. The rapid development of hydrocephalus in this case raises doubts about stroke as a manifestation of SLE. Regarding the pathogenesis of stroke in SLE patients, primary and secondary causes have been suggested. The primary causes include vasculitis, specific antineuronal antibodies and lupus anticoagulants. The secondary causes are renal disorders, hypertension and steroid administration [14].
Although most of the above-mentioned pathogeneses and pathophysiologic mechanisms that can lead to communicating hydrocephalus and other neuropsychiatric manifestations are also predisposing risk factors for stroke which estimated to occur in 5-20% of SLE patients [14]. This wide range of incidence might partly reflect recent advances in neuroradiologic techniques as well as the actual duration of observation. The patient reported in our case had chronic renal failure and treatment resistant hypertension, his SLE had been previously and recently treated with corticosteroids, and he showed evidence of current meningoencephalitis.
The pathogenesis and pathophysiologic mechanisms of communicating-type hydrocephalus in patients with SLE, such as dural sinus thrombosis, hypertension, hyperviscosity, renal failure, infection, immunocomplex deposition in the arachnoid villi, and so forth can also be considered predisposing risk factors for stroke and a provocative pathogenesis of non-communicating hydrocephalus. Ischemic cerebral edema is usually a combination of cytotoxic and vasogenic edema, and there is evidence to suggest that even small infarcts may result in edema that contributes to further ischemia, which appears to contribute heavily to morbidity and mortality in stroke [15,16].

CONCLUSION
We can conclude that multiple pathogeneses and pathophysiologic mechanisms may act in a synergic way in the development of both communicating and non-communicating hydrocephalus. Reviewing the litrature we can realize that our maniscription might be the first to mention about all the pathogenesis and pathophysiologic mechanisms that can lead to communicating hydrocephalus and other neuropsychiatric manifestations are also predisposing risk factors for stroke and subsequently to noncommunicating hydrocephalus. In the case reported here, we theorize that direct compression of the aqueduct of Sylvius by SLE post-inflammatory reactions led to ischemic infarct and brain edema. This resulted in secondary aqueduct stenosis and then to non-communicating hydrocephalus within 48 hours [Fig. 3].
Since hydrocephalus is associated with significant morbidity and mortality, prevention is vital. Early diagnosis of hydrocephalus as part of SLE neuropsychiatric manifestations and detection of its causes may play a key role in the choice of treatment strategy, more accurate patient prognosis and better outcomes. Further researches and studies, such Stereotactic biopsy, postmortem brain and leptomeningeal tissue immunohistochemical investigations are needed to enable scientists interested in this subject to clarify the pathogenesis and pathophysiological mechanisms of the enigma of hydrocephalus associated with SLE.

CONSENT
As per international standard or university standard, patient's written consent has been collected and preserved by the authors.

ETHICAL APPROVAL
All authors hereby declare that all experiments have been examined and approved by the appropriate ethics committee and have therefore been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki.
ACKNOWLEDGEMENT I sincerely thank my parent, family and friends who rendered their spiritual support.