К вопросу дифференциальной диагностики, классификации и дифференцированного лечения гидроцефалии и гидроцефальных синдромов у детей

BackgroundElevated intracranial pressure (ICP) is observed in association with a range of brain disorders. There is limited insight into the regulatory mechanisms of ICP under physiological conditions, and consequently also under pathological conditions. Thereby, to understand the mechanisms underlying ICP dynamics, precise, valid and long-term ICP recordings are of importance in the preclinical setting. Herein, we used a novel telemetric system for ICP recordings which allowed for long-term recordings in freely-moving rats. The aim was to investigate ICP dynamics under different physiological states and investigate how factors such as surgery/recovery, body position, light–dark, co-housing, weight and anesthesia may influence ICP and its waveforms.MethodsA telemetric device was implanted epidurally in rats and signals were recorded continuously for up to 50 days (n = 14). Recording was divided into three experimental periods: a surgical recovery period (RP), a physiological period (PP) and an experimental period (EP). Histology was performed to study the morphology of implanted rats and non-implanted rats (n = 17).ResultsFor the first time, we can demonstrate continuous ICP recordings in freely-moving and co-housed rats for up to 50 days with a high degree of stability. The mean ICP in the recording periods were; RP: 3.2 ± 0.6 mmHg, PP: 5.0 ± 0.6 mmHg and EP: 4.7 ± 0.6 mmHg. In the RP, the ICP was significantly lower compared to the PP (P = 0.0034). Significant light–dark difference in ICP with 21% increase in respiratory slow-wave amplitude was observed in the co-housed animals but not in single-housed animals. The ICP signal was raised during the dark period relative to the light (Δ0.3 ± 0.07 mmHg, P = 0.0043). Administration of anesthesia gave a short-term increase in ICP followed by a significant decrease in ICP. No signs of tissue damage or inflammation were found in the implanted brains.ConclusionsICP dynamics were influenced by several factors such as, use of anesthesia, light–dark difference and housing conditions. Our study demonstrates the importance of performing ICP physiological measurements in freely-moving animals. This has significant implications for moving the preclinical research field forward in order to properly study ICP physiology during disease development and to explore drug targets for alleviating increased ICP.

Intracranial pressure (ICP) is the pressure exerted by the components of the cranial vault, which are the brain, cerebrospinal fluid (CSF), and blood. An elevation of ICP results in a reduction of blood flow to the brain [1]. The brain can cope with intracranial hypertension (ICH) to a certain extent, after which a slight increase in the cerebral volume results in a rapid rise of ICP [2]. A significant reason for death and long-term disability due to head injuries and pathological conditions is an elevation in ICP. An ICP > 20 mmHg is considered a significant threshold and demands an immediate control measure [3]. ICP monitoring can assist in the management of patients with a variety of brain diseases and injuries. The technique has proven valuable, indeed often lifesaving, in the acute care of traumatic brain injury (TBI) [4], hydrocephalus [5], drowning [6], inflammatory and related cerebral diseases such as Reye?s syndrome [7], intracranial hemorrhage [8], and postoperative suboccipital brain tumors [9]. Most patients have headaches and other symptoms suggestive (but not always indicative) of raised ICP; continuous access to ICP levels would greatly facilitate their management. There is a conspicuous need for a wireless implantable ICP-monitoring system as several chronic diseases are associated with ICH. Accurate monitoring of the ICP following a neurosurgical procedure is a basic requirement for adequate treatment [10]?[12]. Since the intracranial contents exist within a rigid vault (the skull), direct ICP measurements require neurosurgical intervention, with its attendant risks. An implant placed during surgery, for the underlying cause of neural disorder, would be a useful adjunct to patient care.

The aim is to investigate the clinical characteristics of systemic lupus erythematosus with intracranial hypertension. The clinical characteristics of one case of systemic lupus erythematosus with chronic persistent intracranial hypertension were analyzed, and related literature was reviewed by searching Medline and Wanfang databases. Intracranial hypertension in SLE patients may occur at the onset or during the course of the disease. Our patient was diagnosed with IH 3years after the onset of SLE. Headache and papilledema were the most common symptoms of intracranial hypertension, followed by nausea or vomiting, vision changes, and cerebral palsy. Our patient had a headache and cranial hypertension that lasted for years, but no papilledema was found. Corticosteroid is currently the mainstay of the treatment of IIH in patients with SLE, and immunosuppressive agents, acetazolamide, intravenous mannitol and furosemide are also used. However, our patient did not respond to these treatments and presents the characteristics of chronic persistent intracranial hypertension. Systemic lupus erythematosus with intracranial hypertension is a rare manifestation of SLE, which is not completely parallel to SLE activity. Headache and papilledema were the most common presenting symptoms. Different from previous reported cases, our patient had poor response to treatments, showing chronic and persistent characteristics.

Intracranial pressure (ICP) was recorded continuously in chronically prepared, unanesthetized cats in order to investigate the effects on ICP of the cholinergic agonist, carbamylcholine (carbachol), injected by microsyringe needles into the dorsal pontine tegmentum. As reported previously, carbachol microinjections into the medial part of the cholinoceptive pontine inhibitory area (CPIA) located ventromedially to the locus coeruleus produced a comatose state characterized by a profound unresponsiveness to external stimuli, desynchronized electroencephalograms (EEG's), and suppression of postural somatomotor and sympathetic visceromotor functions. Four of six ICP records following carbachol microinjections into the CPIA showed small but significant increases which occurred in association with these carbachol effects. Tracings of ICP increases ranged up to 3.2 mm Hg and were similar in shape to plateau waves. The start and resolution of these carbachol-induced ICP variations were closely associated with the onset and termination of EEG desynchronization and signs of reduced cervical sympathetic tone, but not with changes in systemic arterial blood pressure or arterial pCO2. Temporal associations between ICP increases, desynchronized EEG's, and signs of reduced sympathetic tone were repeatedly confirmed during recovery periods associated both with recurrent comatose states following wakefulness produced by various intensities of external stimulation and with spontaneously occurring states resembling rapid eye movement sleep. The authors infer that carbachol-induced ICP variations may be produced by increased cerebral blood volume in response to accelerated cerebral metabolism and reduced vasoconstrictor tone of cervical sympathetic nerves. The simultaneous occurrence of continuously accelerated cerebral metabolism and reduced cervical sympathetic tone can neither be seen in physiologically normal, awake organisms nor produced by other known experimental manipulations of the central nervous system. Such a paradoxical relationship appears to be a unique consequence of activity within the CPIA. These data suggest that episodic activity within the CPIA may provide at least one endogenous neural basis for plateau waves seen during certain pathological conditions such as disturbed cerebrospinal fluid (CSF) absorption or with reduced equilibrium volume of CSF space.

Idiopathic Intracranial Hypertension Presenting With Psychiatric Symptoms

Objective To investigate the clinical, imaging and pathological characteristics of one case of mitochondrial encephalopathy. Methods The data of physical examination, electromyopraphy (EMG), cranial CT and MRI, electroencephalography (EEG), serologic examination, and light microscopy and electron microscopy of muscle biopsy specimens of one case of mitochondrial encephalopathy were collected. The related literatures were reviewed. Results A 52-year-old female patient was admitted with an 8-month of seizures, numbness of the limbs for three times. The clinical manifestations were mainly focal seizures with intelligence disturbance and positive bilateral pyramidal tract sign. The imaging examination was considered to be ischemic stroke and cerebral atrophy. EEG revealed moderate and severe abmormal brain wave. In cerebrospinal fluid (CSF) examination, the intracranial pressure (ICP) was initially 2.01 kPa and finally 1.03 kPa, red blood cell count 1.04 × 10 6/L, white blood cell count 5 × 10 6/L (with an increase in neutrophilic granulocyte proportion), protein 0.70 g/L, chlorides 103.30 mmol/L and IgG 51.90 mg/L. Serum high-sensitivity C-reactive protein (hsCRP) was 15.40 mg/L, and erythrocyte sedimentation rate (ESR) 23 mm/h. Brain biopsy revealed cortical neuron swelling, loss of natural conical shape. The number of capillary increased and astrocytes proliferated reactively. Immunohistochemical examination showed that there were positive Mito granular materials inside the swelling and normal cytoplasm of neurons. The proliferative astrocytes were positive for glial fibrillany acid protein (GFAP). The electronic microscopic examination revealed that a large number of abnormal mitochondria various in shape and size were accumulated between subsarcolemma and myofibrilla. The mitochondrial cristae were flattened or prolonged and twining in concentricity. Osmiophilic bodies and crystalloid inclusion bodies were found in mitochondria. Conclusion Mitochondrial encephalopathy is a rare disease which may be misdiagnosed as ischemic stroke. The diagnosis of mitochondrial disease must be based upon clinical manifestations and laboratory examinations including serum enzymology, biochemistry, molecular biochemistry, muscular biopsy, etc.

Calcified chronic subdural hematoma (SDH) or armoured brain is especially rare with only slight pathophysiology understanding. It happened after head trauma, subdural effusion, meningitis, or as a sequel of VP Shunt. But there is no definite mechanism of neither the pathogenesis nor the management. Because these patients have a thick calcified inner membrane, the optimal surgical procedure for armoured brain’s patient has not been established. Moreover, it is also difficult to obtain good re-expansion of the brain after surgery. A calcified chronic subdural hematoma is less likely happened in adults or elder rather than children or young adults. Management of calcified chronic subdural hematoma is mostly individualized. With seizures, careful monitoring of the anticonvulsant therapy is required. The indications of surgical procedure can be features of raised intracranial pressure, headache, or neurological deterioration. The surgical strategies depend on calcification’s thickness and extension. This study was a preliminary study of Achmad Adam’s research project. This was a descriptive observational research with a case report design. We report a rare case of calcified chronic subdural hematoma in the elderly, presenting with progressive neurological deficit and acute raised intracranial pressure, which was managed surgically to facilitate cerebral re-expansion and inhibit probable brain atrophy. Therefore, authors intended to share a case report regarding a rare chronic subdural hematoma and its management, so hopefully it could be acknowledged by other surgeons as something to learn from.

A simple mathematical model of intracranial pressure (ICP) dynamics is presented. It incorporates the main factors which are known to affect ICP in the short period, i.e., the cerebrospinal fluid (CSF) circulation, the nonlinear craniospinal compliance and the active cerebral blood volume changes induced by autoregulation. The model is used to study the stability of ICP dynamics in pathological conditions. Analysis of model eigenvalues and of bifurcation diagrams reveals that intracranial dynamics becomes unstable in pathological conditions characterized by a decrease in craniospinal compliance and a decrease in CSF outflow. Computer simulations show that the lose of stability manifests itself through a paradoxical ICP response to small initial perturbations and, in the more severe cases, with large-amplitude self sustained waves (plateau waves). All the previous results agree with those reported in the clinical literature.

Results It was agreed that there would be two definitions as seen in all dictionaries. One would be for general use (1) and the second would be for those involved in pathophysiologic studies (2). Definition 1. Hydrocephalus is a dynamic imbalance between the formation and absorption of cerebrospinal fluid resulting in an excessive accumulation of the CSF within the ventricles of the brain. Definition 2 Hydrocephalus is “a dynamic imbalance between the formation and absorption of cerebrospinal fluid resulting in accumulation of excess CSF associated with ventricular dilatation and/or enlargement of the subarachnoid space. The classification would be based on the presence or absence of a point of obstruction and if there is a point of obstruction where that obstruction is located. The various types of hydrocephalus could then be modified by subclassification as to chronicity and as to treatment options. Conclusions Use of a new classification scheme is essential since the latest classification dates to 1914. Use of a classification based on a point of obstruction will lead to a common language among clinicians and researchers which has been absent generally and assure that animal models used in the laboratory can be compared to appropriate clinical conditions.

To evaluate the condition of incontinence in patients with senile dementia, we performed computed tomography X-rays to the brain and analyzed the relationship among the circulatory defect of the brain, the brain atrophy and the degree of incontinence. There were 92 patients subjected to this study who were hospitalised due to senile dementia; 74 patients had vascular dementia, 10 patients had senile dementia of Alzheimer type, and 8 patients had the mixed type. (age ranged: 54-95 years; mean: 80.3 years). The degree of incontinence in these patients varied as follows: 18 patients with continence, 16 patients with moderate incontinence, 58 patients with total incontinence. The diagnosis of circulatory defect of the brain was based on computed tomography observation of periventricular lucency (P.V.L.), and the degree of brain atrophy was evaluated based on 4 criteria: the Lateral body ratio, the Huckman number, the Evans ratio, and the enlargement of the subarachnoid space. Among the 92 patients, P.V.L. was present in 31 patients, among them 27 patients suffered from incontinence. There was a significant correlation between P.V.L. and incontinence (p < 0.001). As the incontinence progressively worsened (Continence, Moderate incontinence Total incontinence), the lateral body ratio increased to 24.8, 27.8, 28.6, (p < 0.05). The Huckman number also increased to 18.3, 19.3, 21.3, (p < 0.01), and the evans ratio likewise 29.9, 32.3, 33.7 (p < 0.01). The enlargement of the subarachnoid space was also correlated with the severity of incontinence. We conclude that urinary incontinence originating from senile dementia is connected to brain atrophy and is strongly influenced by the circulatory disorders of the brain.

The etiological diagnosis of intracranial hypertension is quite complicated but important in clinical practice. Some common causes are craniocerebral injury, intracranial space-occupying lesion, subarachnoid hemorrhage, and hydrocephalus. When a patient presents with intracranial hypertension, the common causes are to be considered first so that other causes would be dismissed. With the morbidity lower than 9%, neuromelanin is very rare. Common symptoms include nerve damage symptoms, epilepsy, psychiatric symptoms, and cognitive disorders. We present a patient with melanoma which manifested with isolated intracranial hypertension without any other neurological signs. A 22-year-old male had repeated nausea and vomiting for 2 mo with Babinski sign (+) on both sides, nuchal rigidity, and subarachnoid hemorrhage. He had been diagnosed with melanoma and was given surgery and whole-brain radiation. Ultimately, the patient died 2 mo later. Malignant melanoma should be taken into consideration in the differential diagnosis of intracranial hypertension.

Slow and rhythmic oscillations in intracranial pressure (ICP), also known as B waves, have been claimed to be one of the best preoperative predictive factors in idiopathic adult hydrocephalus syndrome (IAHS). Definitions of B waves vary widely, and previously reported results must be treated with caution. The aims of the present study were to develop a definition of B waves, to develop a method to estimate the B-wave content in an ICP recording by using computer algorithms, and to validate these procedures by comparison with the traditional visual interpretation. In eight patients with IAHS, ICP was continuously monitored for approximately 20 hours. The ICP B-wave activity as a percentage of total monitoring time (B%) was estimated by using visual estimation according to the definition given by Lundberg, and also by using two computer algorithms (Methods I and II). In Method I each individual wave was classified as a B wave or not, whereas Method II was used to estimate the B-wave content by evaluating the B-wave power in 10-minute blocks of ICP recordings. The two computerized algorithms produced similar results. However, with the amplitude set to 1 mm Hg, Method I yielded the highest correlation with the visual analysis (r = 0.74). At least 5 hours of monitoring time was needed for an acceptable approximation of the B% in an overnight ICP recording. The advantages of using modern technology in the analysis of B-wave content of ICP are obvious and these methods should be used in future studies.

Nimodipine, a Ca2+ antagonist with cerebrovasodilatory and anti-ischemic effects, binds to rat, guinea pig, and human brain membranes with high affinity (less than 1 nM). Only at higher concentrations has nimodipine been reported to block the release of some neurotransmitters and hormones from neuronal tissue. Nimodipine has no consistent effect on brain oxygen consumption or cortical ATP or phosphocreatine levels, although the ischemia-induced fall of brain ATP levels in gerbils or the lowering of intracellular brain pH in rabbits with focal cerebral ischemia were antagonized by the drug. In rats and baboons with middle cerebral artery occlusion, nimodipine was found to reduce neurological deficits without an increase in intracranial pressure or brain edema. Electrophysiological studies with nimodipine suggested a direct neuronal action. In rabbit dorsal root ganglion cells, concentrations as low as 20 nM were reported to block inward Ca2+ currents. Recent studies have suggested that nimodipine may also improve memory in brain-damaged or old rats, restore sensorimotor function and abnormal walking patterns of old rats, and accelerate acquisition of associative learning in aging rabbits. Blockade of age-related changes in Ca2+ fluxes in rat hippocampal neurones by nimodipine in vitro pointed to neuronal plasma membrane as the site of nimodipine action. The therapeutic usefulness of nimodipine appears not to be limited to cerebral ischemia, but may include dementia, age-related degenerative diseases, epilepsy, and ethanol intoxication.

Intracranial pressure (ICP) is a major neurological parameter in animals and humans. ICP is a function of the relationship between the contents of the cranium (brain parenchyma, cerebrospinal fluid, and blood) and the volume of the skull. Increased ICP can cause serious physiological effects or even death in patients who do not quickly receive proper care, which includes ICP monitoring. Epilepsies are a set of central nervous system disorders resulting from abnormal and excessive neuronal discharges, usually associated with hypersynchronism and/or hyperexcitability. Temporal lobe epilepsy (TLE) is one of the most common forms of epilepsy and is also refractory to medication. ICP characteristics of subjects with epilepsy have not been elucidated because there are few studies associating these two important neurological factors. In this work, an invasive (ICPi) and the new minimally invasive (ICPmi) methods were used to evaluate ICP features in rats with chronic epilepsy, induced by the experimental model of pilocarpine, capable of generating the main features of human TLE in these animals.

Mercury poisoning is a rare but fatal toxicologic emergency. Neurologic manifestations involving the central nervous system are seen usually with chronic mercury intoxication. The most commonly seen complaints are headache, tremor, impaired cognitive skills, weakness, muscle atrophy, and paresthesia. Here, we present a male patient who was chronically exposed to elemental mercury and had papilledema and intracranial hypertension without parenchymal lesion in the central nervous system. A 12-year-old male patient was referred to our emergency room because of severe fatigue, generalized muscle pain and weakness, which was present for a month. Physical examination revealed painful extremities, decreased motor strength and the lack of deep tendon reflexes in lower extremities. He had mixed type polyneuropathy in his electromyography. Whole blood and 24-hour urinary mercury concentrations were high. A chelation therapy with succimer (dimercaptosuccinic acid) was started on the fourth day of his admission. On the seventh day of his admission, he developed headache and nausea, and bilateral papilledema and intracranial hypertension were detected on physical examination. Acetazolamide was started and after 1 month of treatment, the fundi examination was normal. The patient stayed in the hospital for 35 days and was then discharged with acetazolamide, vitamin B6, gabapentin, and followed as an outpatient. His clinical findings were relieving day by day. Although headache is the most common symptom in mercury poisoning, the clinician should evaluate the fundus in terms of intracranial hypertension.