Pain and neurological sequelae of cluster munitions on children and adolescents in South Lebanon

This paper aims at evaluating the neurological repercussions arising from injuries sustained due to cluster munitions in children up to 18 years in South Lebanon following the 2006 conflict. Data on neurological and pain symptoms suffered during and after treatment because of sub-munitions in South Lebanon from August 2006 till late 2011 were prospectively recorded. Patients were divided into subcategories; children aged 12 and under and adolescents aged between 13 and 18. During the study period, there were 407 casualties, 122 (30 %) of which were aged 18 years or younger. There were 116 (95 %) males and six (5 %) females. Average age was 14 years. 10 (8.2 %), all males, died as a result of their injuries. 42 (34.4 %) were children and 80 (65.6 %) were adolescents. 112 had surgical treatments for their injuries. 83 out of 112 patients (74 %) with non-lethal injuries had amputations, 67 % children and 78 % adolescents. Among those who had amputations, 31 (37.4 %) suffered from phantom limb pain and 71 % suffered from stump/residual limb pain. 88 % of patients were diagnosed with post-traumatic stress disorder (44 % children and 77 % adolescents) and 41 % were diagnosed with post-concussion syndrome. Four patients (3.6 %) suffered from traumatic brain injuries, both penetrating and closed. Pain syndromes were found in all patients who had amputation. The injury related comorbidities together with many post-concussion syndrome cases, and fewer traumatic brain injuries lead into a high level of physical, psychosocial and economic burdens on the community.     

超早期康复护理对脑梗死患者神经功能肢体功能恢复及情绪改善的影响

目的 探讨超早期康复护理对脑梗死患者神经功能、肢体功能恢复及情绪改善的影响.方法 选取我院2014-01-2016-06收治的脑梗死患者139例,随机分为2组,对照组63例采用常规护理措施,研究组76在对照组的基础上采取超早期康复护理干预措施,在入组时与干预后4周应用中国卒中量表(CSS)评定治疗前后的神经功能,Fugl-Meyer运动功能评估量表(FMA)评定运动功能,改良Barthel指数(MBI)评定日常生活活动能力,SDS、SAS量表分别评定抑郁、焦虑状况.结果 2组入组时CSS评分、FMA评分、MBI评分、SDS评分、SAS评分比较,差异均无统计学意义(P>0.05).干预后4周,2组CSS评分、FMA评分、MBI评分、SDS评分、SAS评分较入组时均明显改善,但研究组改善情况显著优于对照组,差异均有统计学意义(P<0.05).结论 脑梗死患者在常规治疗的同时给予超早期康复护理干预,可显著改善神经功能缺损症状,消除其不良情绪,促进其肢体运动功能的恢复,提升生活质量与预后康复水平.     

The 12 year prognosis of unilateral functional weakness and sensory disturbance

BACKGROUND: Although the symptoms of unilateral "medically unexplained" or "functional" weakness and sensory disturbance present commonly to neurologists, little is known about their long term prognosis. OBJECTIVE: To determine the long term outcome of functional weakness and sensory disturbance. PATIENTS: A previously assembled cohort of 60 patients seen as inpatients by consultant neurologists in Edinburgh between 1985 and 1992 and diagnosed as having unilateral functional weakness or sensory disturbance. METHODS: Current symptoms, disability, and distress were assessed by postal questionnaire to the patients and their family doctors. RESULTS: Follow up data relating to mortality were obtained in 56 patients (93%) and to current diagnosis in 48 patients (80%). Patient questionnaire data were obtained in 42 patients (70%). The median duration of follow up was 12.5 years (range 9 to 16). Thirty five of the 42 patients (83%) still reported weakness or sensory symptoms, and the majority reported limitation of physical function, distress, and multiple other somatic symptoms. Twenty nine per cent had taken medical retirement. An examination of baseline predictors indicated that patients who had sensory symptoms had better functioning at follow up than those who had weakness. Only one patient had developed a neurological disorder which, in hindsight, explained the original presentation. Another patient had died of unrelated causes. CONCLUSIONS: Many patients assessed by neurologists with unilateral functional weakness and sensory symptoms as inpatients remain symptomatic, distressed, and disabled as long as 12 years after the original diagnosis. These symptoms are only rarely explained by the subsequent development of a recognisable neurological disorder in the long term.     

A clinical study of the Guillain-Barré syndrome

60 consecutive patients (age 15-77 years) with the Guillain-Barré syndrome were studied. 37 subjects had an antecedent infection. Onset occurred with motor and/or sensory limb symptoms in 56 cases; 4 subjects experienced onsets with pain, diplopia or bladder disturbances. The motor symptoms reached a maximum within 42 days in all cases, 87% within < 20 days. All patients had limb-muscle weakness at the symptomatic maximum; 50% exhibited cranial-nerve affections and 10 subjects had respiratory insufficiency. Signs of a remission appeared within 60 days after onset in all cases surviving the maximal phase, 81% within < 40 days. The CSF protein concentration was elevated (0.6-7.8 g/l) in 95% of the patients; an increased mononuclear cell count (6-60 × 10⁶/l) occurred in 27% of cases. Electrophysiological abnormalities were detected in 42 out of 43 examined cases.
Totally there were 4 deaths, all caused by cardiac or thrombo-embolic events. Surviving patients had restitutions without functionally significant sequelae within less than 6 months after onset in 73% of cases; all but 2 of the remaining patients recovered within 18 months. The degree of muscle weakness at maximum was the predominant prognostic factor.     

Functional weakness and sensory loss

Functional weakness and sensory loss are common clinical problems with variable presentations. Functional weakness commonly presents as weakness of an entire limb, paraparesis, or hemiparesis, with observable or demonstrable inconsistencies and nonanatomic accompaniments. Documentation of limb movements during sleep, the arm drop test, the Babinski thigh-trunk test, Hoover tests, the Sonoo abductor test, and various dynamometer tests can provide useful bedside diagnostic information on functional weakness. Functional sensory loss typically affects all sensory modalities, either in a hemisensory distribution or affecting an entire limb. Although often inconsistent over serial examinations with nonanatomic features, many clinical findings reported to be helpful in diagnosing functional sensory loss are neither sensitive nor specific for functional sensory loss. The yes-no test, Bowlus-Currier test, and forced-choice tests can provide useful bedside diagnostic information on functional sensory loss. Clinicians must be prepared to make more than one diagnosis in some cases, including an organic neurological diagnosis and a diagnosis of functional overlay. Recent studies have reported relatively low rates (<5%) of misdiagnosis of functional weakness or sensory loss as indicated by subsequent diagnosis of neurological or psychiatric conditions that explained the presenting symptoms. Most neurologists find such patients more difficult to help than patients with organic disease. Management focuses on supportive psychotherapy and behavioral management, exploration of social and psychological issues, treatment of comorbid depression or anxiety, and facilitation of development of more appropriate and constructive coping methods. Many patients with functional weakness, and to a somewhat lesser extent functional sensory loss, have persisting or relapsing-remitting somatic symptoms and persistently impaired social/interpersonal, occupational, and psychological functioning.     

Unusual manifestations of herpes zoster. A clinical and electrophysiological study.

The literature on complicated herpes zoster is summarized in this paper. The case histories of 18 patients with herpes zoster are presented. Two patients had encephalitis, 2 had myelitis and the other 14 patients had various types of lower motor neurone disturbance. Both patients with encephalitis--one of who developed choreo-athetosis during the illness--recovered fully. Only 1 of the 2 patients with myelitis recovered fully; the other remains severely paraparetic and the reason for her incomplete recovery may be related to the presence of generalized arteriolar disease associated with seronegative rheumatoid disease. One patient developed a Guillain-Barre syndrome 3 weeks after the onset of herpes zoster. Recovery in the 15 patients with lower motor neurone involvement has been slow butcomplete--or almost complete--in all but 1, a patient with persistent facial weakness as part of the Ramsay Hunt syndrome and who also had weakness of one upper limb. Seven other patients had lower limb weakness. In 2 patients the weakness was confined to abdominal myotomes and 2 other patients had urinary retention. Electromyographic abnormalities were found in the muscles which were weak and frequently also in muscles which appeared strong. It is emphasized that neurological disturbances other than sensory abnormalities may be found in patients with herpes zoster. Motor complications of various types are not uncommon.     

Myopathies presenting with head drop: Clinical spectrum and treatment outcomes

Dropped head syndrome can be the presenting feature of a wide spectrum of neurological conditions. In this study, we aimed to define the clinical characteristics and treatment outcomes of 107 patients, where head drop was the presenting or predominant clinical feature of a myopathy. Median age at presentation was 68 years (range 42–88). A specific diagnosis was reached in 53% of patients: Inflammatory myopathy (n = 16), myopathy with rimmed vacuoles (n = 10), radiation-induced myopathy (n = 8), sporadic late-onset nemaline myopathy (n = 7), myofibrillar myopathy (n = 4), facioscapulohumeral dystrophy (n = 3), inclusion body myositis (n = 2), mitochondrial myopathy (n = 2), scleroderma-associated myopathy (n = 2), and single cases of necrotizing autoimmune myopathy, drug-induced myopathy, and B-cell chronic lymphocytic leukemia-myopathy. Splenius capitis had the highest diagnostic yield for a muscle biopsy (67%). When tested, 31/35 (89%) of patients had abnormal pulmonary function tests, 15/30 (50%) abnormal swallow evaluation, 24/65 (37%) abnormal electrocardiogram and 5/38 (13%) abnormal transthoracic echocardiogram. 23/43 (53%) treated patients responded to treatment. Patient-reported limb weakness and neck flexion weakness on physical examination were associated with good response to treatment. A wide spectrum of acquired and hereditary myopathies can present with head drop, some of which are potentially treatable. Establishing a diagnosis is crucial for timely treatment administration, screening for swallowing and cardiorespiratory involvement, and counseling regarding prognosis.     

Neuromuscular manifestations of critical illness

Critical illness, more precisely defined as the systemic inflammatory response syndrome (SIRS), occurs in 20%-50% of patients who have been on mechanical ventilation for more than 1 week in an intensive care unit. Critical illness polyneuropathy (CIP) and myopathy (CIM), singly or in combination, occur commonly in these patients and present as limb weakness and difficulty in weaning from the ventilator. Critical illness myopathy can be subdivided into thick-filament (myosin) loss, cachectic myopathy, acute rhabdomyolysis, and acute necrotizing myopathy of intensive care. SIRS is the predominant underlying factor in CIP and is likely a factor in CIM even though the effects of neuromuscular blocking agents and steroids predominate in CIM. Identification and characterization of the polyneuropathy and myopathy depend upon neurological examination, electrophysiological studies, measurement of serum creatine kinase, and, if features suggest a myopathy, muscle biopsy. The information is valuable in deciding treatment and prognosis.     

A Guillain-Barré syndrome variant with prominent facial diplegia

To determine the clinical features of a Guillain-Barré syndrome variant with prominent facial diplegia, we retrospectively reviewed approximately 8,600 cases referred to our neuroimmunological laboratory for serological tests during the past seven years. Patients’ histories, neurological signs, and laboratory and electrophysiological data were clarified based on their clinical records. Sera obtained during the acute phase were tested for prior infectious serology and anti-ganglioside antibodies. In 22 patients, clinical signs such as acute progressive bifacial weakness, paresthesias in the distal dominant limbs, and hypo- or areflexia, were compatible with a Guillain-Barré syndrome variant, facial diplegia and paresthesias. Other cranial nerve involvements, limb weakness, and ataxia were absent or minimal. Clinical courses were monophasic, the nadir being reached within four weeks. Eighteen patients (86%) had had infectious symptoms within the four weeks preceding the onset of neurological illness. In the infection serology tests, anti-cytomegalovirus IgM antibodies were the most frequent (35%). All the patients had cerebrospinal fluid albuminocytologic dissociation. In nerve conduction studies, 14 (64%) showed demyelination in their limbs. Anti-GM2 IgM antibodies were detected in four patients who had anti-cytomegalovirus IgM antibodies. Patients with conditions similar to facial diplegia and paresthesias, but lacking either distal paresthesias or hyporeflexia, were regarded as having marginal facial diplegia and paresthesias, because they also frequently had features of Guillain-Barré syndrome, such as an antecedent infection or cerebrospinal fluid albuminocytologic dissociation. Our findings are further evidence of a facial variant of Guillain-Barré syndrome and provide important information essential for its diagnosis.     

Morbidity predictors in ischemic stroke

BACKGROUND: Although ischemic CVA is one of the leading causes for death and disability, parameters for predicting long-term outcome in such patients have not been clearly delineated, especially in the Indian context. METHODS: A prospective hospital-based study of 105 patients of ischemic stroke, focal neurological deficits and functional score was assessed and the C-reactive protein level (CRP) was measured. A follow-up was done at 5 days and at 6 months and outcome variable was the functional status at 6 months using Barthel Index of Activities of Daily Living. Accordingly, patients were grouped into 3 - Barthel Index < 41: Severely disabled, Barthel Index 41-60: Moderately disabled and Barthel Index > 60: Mildly disabled. RESULTS: At admission, if upper limb power was less than Medical Research Council (MRC) grade 4, or aphasia was present or CRP assay was positive, then at 6 months, these patients most likely belonged to the severely disabled group. If upper limb or lower limb power was greater than MRC grade 3 or there was no aphasia or conjugate gaze deviation or CRP assay was negative, these patients most likely belonged to the mildly disabled group at 6 months. Follow-up rate was 86%. CONCLUSION: Patients can be stratified according to the predicted prognosis. The treatment and rehabilitation can be properly planned and strictly adhered to in patients predicted to have worse prognosis.     

Community walking activity in neurological disorders with leg weakness

BACKGROUND: The aims of this study were to determine walking mobility in the community in individuals with lower limb weakness and to establish the extent to which some clinic based measures predict such activity. METHODS: Five groups (n = 12-18) of independently ambulant patients with lower limb weakness due to neurological conditions and a matched healthy control group were recruited. Measures of isometric knee extension/flexion muscle strength, time to stand up (sit-to-stand, STS), gait speed, and daily step counts (recorded over 7 days) were obtained. The Rivermead Mobility Index (RMI) provided a measure of functional ability. Between group differences and associations were explored. Backward stepwise regression analysis was used to identify variables influencing daily step count in individuals with neurological impairment. RESULTS: Patients were significantly weaker (mean (SD) quadriceps strength 69+/-34% v 102+/-37% predicted), slower to stand up (2.9+/-1.3 v 2.0+/-0.6 s), and had slower self selected gait speed (0.74+/-0.3 v 1.2+/-0.2 m/s) than controls. Mean daily step count was also lower (3090+/-1902 v 6374+/-1819) than in controls. In neurology patients step count was correlated with RMI score (r(s) = 0.49, p<0.01) and STS (r = -0.19, p<0.05). However, self selected gait speed was the only significant predictor in the regression analysis (p<0.01) of daily mean step count. CONCLUSIONS: Measures of muscle strength, timed STS, and RMI do not appear to closely reflect community walking activity in these patient groups. Self selected gait speed was partially predictive. Measurement of community walking activity may add a new dimension to evaluating the impact of interventions in neurological disorders.     

Functional neurological disorders in outpatient practice: An Australian cohort

Functional disorders are defined as neurological symptoms without causative organic pathology identified. They are a diverse and often neglected group of disorders. The aim of this was to determine the incidence and outcome of functional neurological disorders in an Australian neurology practice. Over a 17 month period, all patients presenting to a single outpatient neurology service were evaluated to determine the incidence and outcome of these disorders. A total of 884 patients were assessed and of these, 137 had a final diagnosis of functional neurological illness, equating to an incidence of 15% of all patients seen. Functional disorders were the third most common presentation overall. Patients with functional disorders were younger, more likely to be female and had a higher rate of current psychiatric comorbidity compared to other neurology patients. Sensory symptoms were the most common manifestation (48%) followed by limb weakness (37%) and psychogenic non-epileptic seizures (14%). Outcome information was available for 49% of patients at an average of 3 months follow-up. 45% had some improvement in their symptoms, 43% had static symptoms and 12% had worsening of symptoms. This study confirms the high incidence of functional disorders in outpatient neurology practice. Early improvement was seen in a substantial proportion of patients and is influenced by duration of symptoms.     

Prediction of disease progression in Miller Fisher and overlap syndromes

Patients with Miller Fisher syndrome (MFS) may have a relatively mild clinical course or progress to Guillain‐Barré syndrome (GBS) with limb weakness (MFS‐GBS overlap syndrome). Other variants in this spectrum are GBS with ophthalmoparesis and Bickerstaff's Brainstem encephalitis (BBE). To compare the clinical course of MFS and overlap syndromes and to identify predictors of disease progression. In a prospective study of 170 patients with GBS and variant forms, 37 (22%) had a MFS, MFS‐GBS overlap syndrome, ophthalmoplegic GBS or BBE. The clinical, serological, and electrophysiological features were compared. Twenty‐three patients presented with MFS, of which 10 (43%) developed limb weakness (MFS‐GBS overlap syndrome). All these transitions occurred in the first week after onset of symptoms. There were no differences in the clinical, electrophysiological and serological features at entry between MFS and MFS‐GBS. Twelve patients had ophthalmoplegic GBS and the disease severity at nadir and outcome was worse than in the patients with a MFS‐GBS overlap syndrome. No early predictors for progression from MFS to MFS‐GBS overlap syndrome were found. All transitions occurred in the first week. This finding implicates that all patients with MFS need careful monitoring for at least 1 week.     

Calgary experience with West Nile virus neurological syndrome during the late summer of 2003

BACKGROUND: Between August 25 and September 25, 2003 seven patients with West Nile virus neurological manifestations were identified through the hospital neurology consultation services in Calgary, Alberta, Canada. Three of the seven patients were treated with interferon alpha-2b (IFN alpha-2b). In this report we document the clinical characteristics of these seven cases. METHODS: Clinical and laboratory information was obtained from a retrospective review of patient hospital and clinic charts. Patients were included if they had serological evidence of West Nile virus infection and had clinical evidence of aseptic meningitis, encephalomyelitis, cerebellar syndrome or motor neuronopathy. Three patients received a treatment course of three million units IFN alpha-2b, administered by subcutaneous injection once per day for 14 days. RESULTS: Four patients had cerebellar signs without change in consciousness, two had both encephalitis and neuromuscular weakness, and one patient had focal lower motor neuron arm weakness. The mean age was 52 (range 24 - 73). All patients had flu-like illness and fever as presenting symptoms and six had severe headaches. Two patients were immunocompromised prior to infection. Two patients with cerebellar signs (one with opsoclonus-myoclonus) improved spontaneously and exhibited only mild residual deficits on discharge. The other two patients with cerebellar findings developed brainstem involvement, one coinciding with and one subsequent to the cerebellar symptoms. Within one week of treatment with IFN alpha-2b these latter two patients showed marked improvement. One patient with encephalitis and neuromuscular weakness, was treated with IFN alpha-2b and subsequently recovered. INTERPRETATION: In this case review of seven patients, multiple neurological symptoms occurred in each patient and the neurological presentation was varied. Four patients had predominant cerebellar findings and one patient had opsoclonus-myoclonus, not previously reported. The marked improvement in three patients who received IFN alpha-2b raises preliminary optimism towards this potential treatment.     

Late onset Pompe disease: clinical and neurophysiological spectrum of 38 patients including long-term follow-up in 18 patients

To describe the clinical and neurophysiological spectrum and prognosis in a large cohort of biochemically and genetically proven late onset Pompe patients. Thirty-eight diagnosed with late onset Pompe disease at our neuromuscular department during 1985 and 2006 are described in detail. The mean delay from onset of symptoms or first medical consultation until diagnosis was 10.4 and 7.1 years, respectively. A different diagnosis was suggested in 11 of 38 patients. Ten patients underwent repeated muscle biopsies before diagnosis of Pompe disease was established. Limb girdle weakness was the most frequent presenting sign. Six patients complained of myalgia. Wolf-Parkinson-White syndrome was found in 3 of 38 patients. Respiratory failure preceded the onset of overt limb muscle weakness in three patients. The course of the patients was progressive in all, but there was a wide variety of progression, which did not correlate with the age of disease onset. In 71% of the patients, neurophysiological investigations revealed a myopathic EMG pattern, half of the patients had spontaneous activity including complex repetitive discharges. A normal EMG was found in 9% of the patients. Nerve conduction studies were normal in all. Pompe disease should be taken into consideration in patients with unexplained limb girdle muscular weakness with respiratory failure. Cardiac manifestations may not be restricted to infantile Pompe disease.     

Difference in distribution of muscle weakness between myasthenia gravis and the Lambert-Eaton myasthenic syndrome

BACKGROUND: Myasthenia gravis and the Lambert-Eaton myasthenic syndrome (LEMS) may have a similar distribution of muscle weakness. Deciding on a diagnosis of myasthenia gravis or LEMS on clinical grounds may therefore be difficult. OBJECTIVE: To compare the localisation of initial muscle weakness and the distribution of weakness at the time of maximum severity in patients with myasthenia gravis and LEMS. SUBJECTS: 101 patients with myasthenia gravis and 38 patients with LEMS. RESULTS: In myasthenia gravis, initial weakness involved extraocular muscles in 59%, bulbar muscles in 29%, and limb muscles in 12% of the patients. In LEMS no patient had ocular weakness, 5% had bulbar weakness, and 95% had weakness of the limbs as the first symptom (p < 0.001). At the point of maximum severity, weakness in myasthenia gravis was purely ocular in 25%, oculobulbar in 5%, restricted to the limbs in 2%, and present in both oculobulbar muscles and limbs in 68%. At this point, none of the LEMS patients had weakness restricted to extraocular or bulbar muscles (p = 0.002). The legs were affected in all LEMS patients, whereas in 12 patients with generalised myasthenia gravis limb weakness was restricted to the arms (p = 0.024). CONCLUSIONS: In a patient suspected to have a myasthenic syndrome whose first symptom is ocular weakness, LEMS is virtually excluded. Limb weakness confined to the arms is only found in generalised myasthenia gravis and not in LEMS. Muscle weakness in myasthenia gravis tends to develop in a craniocaudal direction, and in the opposite direction in LEMS.     

Treatment and outcome of myasthenia gravis: retrospective multi-center analysis of 470 Japanese patients, 1999-2000

To clarify the current status of treatments and outcomes of patients with myasthenia gravis (MG) in Japan, a total of 470 patients (164 men and 306 women; mean age 41 years) were recruited from 19 Japanese tertiary medical centers in 1999-2000. Thymectomy was performed in 319 (68%) of the patients. Patients who received thymectomy were younger (p = 0.01) and had more severe disabilities (p < 0.01) than patients without thymectomy. Irrespective of receiving thymectomy, most of the patients were administered corticosteroids (64%), other immunosuppressive agents (10%), or cholinesterase inhibitors (86%). Of 395 patients followed up for more than 12 months after treatment (mean 8.0 years), 30% (34% of thymectomized and 21% of non-thymectomized patients) were in remission (no symptoms with/without medication), 34% had only ocular symptoms, and the remaining 35% still had weakness of bulbar or limb muscles at the end of follow-up. The prognosis of MG in Japan was generally favorable, but despite the frequent use of thymectomy and immunosuppressive treatments, approximately one-third of patients still had generalized weakness. More effective or intensive treatments are required to improve the prognosis.     

Exchange transfusion: a low-cost alternative for severe childhood Guillain-Barré syndrome

The high cost and nonavailability of plasmapheresis and intravenous immunoglobulin are prohibitive for the treatment of Guillain-Barré syndrome in resource-poor settings. Exchange transfusion can be an alternative therapy for severe disease in children. The effectiveness of exchange transfusion was evaluated in nine children (median age 6 years) with severe Guillain-Barré syndrome (functional disability score >/= 4). All patients had lower limb weakness for a median duration of 4 days (range 2-16 days). Upper limb weakness (n = 8), respiratory involvement (n = 7), pooling of oral secretions (n = 3), and facial palsy (n = 1) were other motor signs. Four children developed respiratory failure; three were ventilated. Other clinical features included leg pains, meningismus and Lasàgue's sign, backache, excessive sweating, and hemodynamic instability. Two children had albuminocytologic dissociation on cerebrospinal fluid analysis. Nerve conduction velocity testing was done in three patients; two had suggestive findings. One course of exchange transfusion could be performed in seven patients. Six children (86%) responded well with one or more of the following: a halt in progressive motor weakness, improvement in leg pains, meningismus, and straight-leg raising within 24 to 48 hours; one could be weaned off the ventilator by 60 hours. All had improvement in motor power of at least one muscle group by one grade within 3 to 7 days. Two patients died, whereas five (71% of the treatment group) were discharged and could walk independently by 4 months. Two untreated ones had died. Exchange transfusion seems to be a safe and effective alternative for severe Guillain-Barré syndrome. It should be offered whenever intravenous immunoglobulin or plasmapheresis is not available or affordable. Replacement of immunosaturated red cells, removal of activated T cells and monocytes, and transient thrombocytopenia are possible explanations for its effectiveness in addition to removal of pathogenic autoantibodies, immune complexes, cytokines, and complements.     

Severe acute Guillain-Barré syndrome

Six of 58 consecutive patients with Guillain-Barré syndrome had an acute, severe, and prolonged initial illness, with quadriplegia in 2 to 5 days and mechanical ventilation for over 2 months. The average times in the ICU, on a ventilator, in the hospital, and in rehabilitation were 62, 141, 157, and 148 days, respectively. Four were still bedbound and ventilated at 6 months. Three (5%) were limited to a chair, and three walked unsteadily or required foot splints 2 to 3 years after onset. Only 2 of 13 other ventilated patients with slower initial progression of weakness, and none of 38 nonventilated patients were chairbound 6 months after onset (1 died at 2 months); all were walking independently by a year. Quadriplegia appearing over 2 to 5 days is associated with the most severe and prolonged weakness and, in some patients, leads to a permanent chairbound state. Improvement stops at 1 1/2 to 2 years.     

Critical illness polyneuropathy and myopathy: clinical features, risk factors and prognosis

Acquired neuromuscular weakness due to critical illness polyneuropathy and myopathy (CIPNM) frequently develops in patients hospitalized in the intensive care unit for more than 1 week. CIPNM may present with muscle weakness and failure to wean from mechanical ventilation, but is discovered more often and earlier by electrophysiological examination. In this review, the incidence, clinical and electrophysiological features, differential diagnosis and prognosis of CIPNM will be described. Risk factors for CIPNM are sepsis or systemic inflammatory response syndrome and the severity of multi-organ failure. Presence of CIPNM is associated with higher mortality rate, prolonged duration of mechanical ventilation and prolonged rehabilitation. The majority of survivors with CIPNM have persistent functional disabilities and a reduced quality of life. There is need for new therapeutic strategies to prevent or minimize CIPNM in critically ill patients.