Neurological disorders in the intensive care unit

Neurological problems are common among critically ill patients; they often signal that other organs are failing, but are themselves important causes of morbidity and mortality. Cognitive function may suffer as a consequence of septic encephalopathy, the pathophysiology of which is poorly understood; however, the affected patients usually return to their baseline when sepsis resolves. Seizures and cerebrovascular disorders are also common in the intensive care unit. Neuromuscular complications are important causes of failure to wean from mechanical ventilation and lead to substantial long-term morbidity.     

Useful parameters helping proctologists to identify patients with defaecatory disorders that may be treated with pelvic floor rehabilitation

Background No studies have specifically reported on the use of a diagnostic tool based on physiatric assessment of constipated or incontinent patients Methods Sixty-seven constipated and 37 incontinent patients were submitted to a standard protocol based on proctologic examination, clinico-physiatric assessment (puborectalis contraction, pubococcygeal test, perineal defence reflex, muscular synergies, postural examination) and instrumental evaluation (anorectal manometry, anal US and dynamic defaecography). Patients were offered pelvic floor rehabilitation (thoraco-abdominoperineal muscle coordination training, biofeedback, electrical stimulation and volumetric rehabilitation). Results After rehabilitation treatment, decreases of Wexner constipation score (p=0.0001) and Pescatori incontinence score (p=0.0001) were observed. Conclusion This diagnostic protocol might improve the selection of patients with defaecatory disorders amenable for rehabilitation treatment.     

Neurological disorders in essential thrombocythemia

Patients with essential thrombocythemia often complain of various subjective neurological symptoms. This prospective study aims to assess their incidence and response to therapy. Among 37 consecutive patients with essential thrombocythemia, 11 presented with neurological symptoms. Among them 4 had thrombotic events, 7 complained of transient or fluctuating subjective symptoms, and one had both. Brain magnetic resonance imagery failed to detect any substratum in patients with subjective symptoms. JAK2V617F mutation was found in 9 of 11 patients with neurological symptoms versus 14 of 26 patients without symptoms. Ten patients received low-dose aspirin for these symptoms: complete resolution was observed in 3, improvement with persisting episodes in 2, and resistance to aspirin in 2 patients, in whom addition of cytoreductive therapy became necessary to resolve those disabling symptoms. In this prospective cohort, 30% of patients with essential thrombocythemia presented neurological symptoms. Aspirin was fully efficient in only 30% of cases. JAK2V617F mutation could be a risk factor for such symptoms.     

Effect of intensive physical training on the hospital stage of the rehabilitation of patients with macrofocal myocardial infarct

Thirty-five patients with large-focal myocardial infarction were divided into the main (21) and the control (14) groups. The main-group patients were subjected to intensive exercise beginning with day 10-22 of the disease; its program was designed so as to match the data of bicycle ergometry checkups and included bicycle-ergometric riding and rationed walking. It was demonstrated that the main group patients showed better physical stress tolerance, improved myocardial functional potentials, better psychological outlook and smaller pulmonary venous congestion at the time of transfer to the sanatorium stage of rehabilitation. It is suggested that exercise be incorporated in the hospital rehabilitation complex for patients with large-focal myocardial infarction.     

Peripheral nerve functions may deteriorate parallel to the progression of microangiopathy in diabetic patients

Our aim was to obtain information to help in the early detection of impaired nerve function and to assess the severity of diabetic symmetric polyneuropathy (DPN). Various somatic and autonomic nerve functions in 40 diabetics and 20 age-matched healthy volunteers were evaluated using six objective examinations: nerve conduction study, quantitative vibratory perception threshold, heart rate variability, Valsalva test, head-up tilt and quantitative sudomotor axonal reflex test (QSART). The diabetics were divided into three groups according to the severity of their microangiopathy. The nerve function data and level of impairment were compared between a healthy control and three diabetic groups. The relationships between nerve function data and clinical background were also examined using multivariate analysis. Results were as follows: (1) all nerve dysfunctions seemed to develop parallel to the progression of microangiopathy, (2) reduced nerve conduction velocity and elevated vibratory perception thresholds in the feet might be early detectable signs of DPN, (3) vasomotor and sudomotor sympathetic functions and cardiovagal functions seemed to deteriorate with the appearance of microangiopathy, (4) lowered compound muscle action potential seemed to appear at the advanced microangiopathic condition, (5) hypohydrosis was closely related to diabetic foot ulcers. In conclusion, nerve dysfunction in diabetics might generally progress with microangiopathy from somatic sensory nerve dysfunction to autonomic nerve dysfunction and then to somatic motor nerve dysfunction. Sympathetic sudomotor dysfunction might be a sensitive predictor of diabetic foot ulcer.     

Neurological disorders and adult coeliac disease

An investigation into the incidence of neurological disorder in 30 patients with adult coeliac disease has shown that three patients had severe depression, two had epileptiform convulsions, and one patient only had signs of involvement of the peripheral nervous system. Motor nerve conduction velocity was low in only one patient. When the nerve conduction velocities of the group on a gluten-free diet were compared with the group who were not on a gluten-free diet, there was no statistically significant difference. Similarly, nerve conduction velocities in patients with a low serum pyridoxal level were not significantly different from those with normal serum pyridoxal levels.Measurements of the serum level of pyridoxal in 30 patients confirmed that pyridoxine deficiency occurs in adult coeliac disease and that the restriction of gluten from the diet appeared to affect pyridoxal levels favourably.     

Neurological implications of urea cycle disorders

Summary The urea cycle disorders constitute a group of rare congenital disorders caused by a deficiency of the enzymes or transport proteins required to remove ammonia from the body. Via a series of biochemical steps, nitrogen, the waste product of protein metabolism, is removed from the blood and converted into urea. A consequence of these disorders is hyperammonaemia, resulting in central nervous system dysfunction with mental status changes, brain oedema, seizures, coma, and potentially death. Both acute and chronic hyperammonaemia result in alterations of neurotransmitter systems. In acute hyperammonaemia, activation of the NMDA receptor leads to excitotoxic cell death, changes in energy metabolism and alterations in protein expression of the astrocyte that affect volume regulation and contribute to oedema. Neuropathological evaluation demonstrates alterations in the astrocyte morphology. Imaging studies, in particular ¹H MRS, can reveal markers of impaired metabolism such as elevations of glutamine and reduction of myoinositol. In contrast, chronic hyperammonaemia leads to adaptive responses in the NMDA receptor and impairments in the glutamate–nitric oxide–cGMP pathway, leading to alterations in cognition and learning. Therapy of acute hyperammonaemia has relied on ammonia-lowering agents but in recent years there has been considerable interest in neuroprotective strategies. Recent studies have suggested restoration of learning abilities by pharmacological manipulation of brain cGMP with phosphodiesterase inhibitors. Thus, both strategies are intriguing areas for potential investigation in human urea cycle disorders. Competing interests: None declared     

Neurological disorders and inflammatory bowel diseases

Extraintestinal manifestations occur in about one-third of patients living with inflammatory bowel disease (IBD) and may precede the onset of gastrointestinal symptoms by many years. Neurologic disorders associated with IBD are not frequent, being reported in 3% of patients, but they often represent an important cause of morbidity and a relevant diagnostic issue. In addition, the increasing use of immunosuppressant and biological therapies for IBD may also play a pivotal role in the development of neurological disorders of different type and pathogenesis. Hence, we provide a complete and profound review of the main features of neurological complications associated with IBD, with particular reference to those related to drugs and with a specific focus on their clinical presentation and possible pathophysiological mechanisms.     

Neurological disorders in a rural Western Australian population

Background: There is limited information on the causes of neurological disorders in an Australian rural setting. Methods: This study reports on a prospective cohort of 160 patients (95 women and 65 men) with a mean age of 55.1 years (standard deviation 19.78 years, range 12–92 years) receiving a neurological work‐up from one neurologist attending the Geraldton and Midwest region of Western Australia over a 12‐month period. Results: Patients were divided into 15 diagnostic classifications. Movement disorders were the most common diagnostic classification (38 of 160 or 23.75%) and Parkinson's disease was the most common movement disorder (30 of 38 or 78.95%) with an estimated period of prevalence of 187.5 persons per 1000. Of the other neurological disorder diagnosis classifications the following number of patients per group was observed: epilepsy (27 or 16.87%); neuromuscular disorders (22 or 13.75%); multiple sclerosis (12 or 7.5%); cerebrovascular disease (10 or 6.25%); headaches (7 or 4.37%); neurodegenerative (7 or 4.37%); dementias (6 or 3.75%); memory dysfunction (6 or 3.75%); gait disorders (4 or 2.5%); vestibular syndrome (3 or 1.87%); pain syndrome (3 or 1.87%); sensory syndrome (2 or 1.25%); brain injury (1 or 0.62%) and miscellaneous (12 or 7.5%). A high number of persons having Parkinson's disease was found. Conclusion: Possible risk factors for Parkinson's disease for individuals living in Geraldton and Midwest region need to be investigated. This study raises issues pertaining to the provision of services and allocation of resources in rural areas, especially for patients with Parkinson's disease.     

Neurological aspects of multiple myeloma and related disorders

The spectrum of neurologic complications of multiple myeloma (MM) and related conditions is as diverse as the conditions themselves. Complications range from direct compression (radiculopathy, spinal cord compression, base-of-the-skull tumor) to the infiltrative (amyloid, peripheral neuropathies, and numb chin syndrome of myeloma), the metabolic (slowed mentation from hyperviscosity, hypercalcemia, or uremia), and to autoimmune or cytokine-mediated (peripheral neuropathy). The two most common presentations are the compressive radiculopathy one sees in multiple myeloma and the peripheral neuropathies associated with many of the other disorders. The authors will review the neurologic complications of MM, monoclonal gammopathy of undetermined significance (MGUS), Waldenstr?m macroglobulinemia (WM), POEMS syndrome, amyloidosis, and cryoglobulinemia.     

Neurological disorders associated with cassava diet: a review of putative etiological mechanisms

Tropical ataxic neuropathy (TAN) and epidemic spastic paraparesis (konzo) are two neurological disorders associated with the consumption of cassava (Manihot esculenta) in several African countries. TAN is characterized by sensory polyneuropathy, sensory ataxia, bilateral optic atrophy and bilateral sensori-neural deafness. It occurs in elderly individuals subsisting on a monotonous cassava diet with minimal protein supplementation. Konzo is a syndrome of symmetrical spastic paraparesis with a predilection for children and young women and invariably associated with consumption of inadequately processed bitter cassava roots with minimal protein supplementation. Despite numerous epidemiological, clinical and biochemical studies aimed at elucidating the etiological mechanisms of these disorders, their etiologies remain unknown, and there is no known treatment. The diseases continue to be prevalent in endemic areas, causing significant disability and increased mortality. A fresh appraisal of the putative etiologic mechanisms proposed for these intriguing and enigmatic syndromes is presented in this paper. Evidences against a causal role for cyanide intoxication are discussed, and evidences implicating thiamine deficiency as a unifying etiological mechanism for these neurological syndromes are presented. It is concluded that urgent research is needed to evaluate thiamine status and implement a therapeutic trial of thiamine in these debilitating neurological disorders.     

Neurological and cognitive disorders after coronary artery bypass grafting

Cerebral injury is a major cause of mortality and morbidity of coronary artery bypass grafting. Stroke occurs in 3% of patients and is largely caused by embolization of atheromatous debris during manipulation of the diseased aorta. Cognitive impairment, which is predominantly caused by microembolization of gaseous and particulate matter, mainly generated by cardiotomy suction, is more common. Demonstration of similar cognitive impairment in patients operated on without cardiopulmonary bypass indicates that other pathophysiological mechanisms, such as anaesthesia and hypoperfusion, are also involved. Advances in medical, anesthetic, and surgical management have resulted in a reduction in the incidence of neurological injury in CABG patients over the past decade. On the other hand, an increasingly elderly population with more severe comorbidity, who are more prone to cerebral injury, are increasingly being referred for CABG. Possible mechanisms to reduce overt and subtle cerebral injury are discussed. The use of composite arterial grafts performed on the beating heart may be the most effective way of minimizing the risk of cerebral injury associated with CABG.