[Alexia without agraphia; not being able to read what you have just written]. / Alexie zonder agrafie: niet kunnen lezen wat je zojuist hebt geschreven.

BACKGROUND: Alexia without agraphia is a neurological syndrome that is caused by a specific lesion in the left (or the dominant) cerebral hemisphere. It is characterised by a severe reading disorder with writing ability intact. CASE DESCRIPTION: We describe a patient who, after a fall from a staircase, could no longer read, even that which he had written shortly before. Initially, he also had problems with orientation and facial recognition. Two months after the accident, alexia without agraphia was still manifest while the other symptoms had disappeared. His ability to read showed a slow improvement; complete recovery occurred after two years. The absence of concomitant disorders was exceptional. CONCLUSION: The disorder alexia without agraphia seems improbable, but deserves serious attention to prevent diagnostic delay.

Psychosocial dysfunction in the first year after Guillain-Barré syndrome.

In this investigation we study the impact of Guillain-Barré syndrome (GBS) on psychological distress, depressive symptoms, and health status of patients during the first year after GBS. At 3, 6, and 12 months, patients were given the General Health Questionnaire, the Sickness Impact Profile, and the Center for Epidemiologic Studies Depression Scale. Eighty-five patients participated. Psychological distress and depressive symptoms were present but improved between 3 and 6 months. At 12 months the psychosocial health status was still impaired. Patients who perceived their physical residua to be moderately to seriously disruptive and patients with muscle ache and cramps had worse scores on all scales. It can be concluded that most of the improvement occurred in the first 6 months. Psychosocial health status, however, was still impaired at 1 year, but depressive symptoms played no role. Treatment of muscle ache and cramps, and the disruptive effect of physical residua should be seriously considered.

The effects of Guillain-Barré syndrome on the close relatives of patients during the first year.

OBJECTIVE: To study the impact of Guillain-Barré Syndrome (GBS) on the psychosocial functioning of the closest relative and on family functioning during the first year after GBS. METHOD: At 1 (=T1), 3 (=T3), 6 (=T6), and 12 months (=T12) after the onset of GBS, relatives of patients received the General Health Questionnaire (GHQ28) and the Family Assessment Device (FAD). Sixty-three relatives returned the GHQ28 at all four designated intervals. At T1 the relatives also received a questionnaire that contained questions on the impact on their daily life. The answers to these questions yielded a Daily Living Impact index. From the 110 relatives, 86 returned this questionnaire. RESULTS: 72% of the 86 relatives reported one or more problems in daily living. At T1 the scores of the GHQ subscales ranged from normal to mildly disturbed. The relatives showed significant improvement in their somatic complaints and anxiety during the first half year. Social dysfunction remained somewhat less than normal, severe depression was not found. At T1 and T3 the scores of the GHQ28 and some subscales differed significantly depending on the severity of the functional status of the patient, but not at T6 and T12. Relatives of patients with severe residua at 1 month score worse on the GHQ28 and most subscales at 6 months. The FAD was normal at all moments measured. CONCLUSIONS: Psychological morbidity of close relatives is significantly higher in the first months after the onset of GBS. The patient's condition has an important impact on the psychosocial functioning of close relatives. Therefore, a family approach is recommended to neurologist and other medical personnel during the first period of the disease. Also patient support groups may play a beneficial role for the relatives of GBS patients.

How Guillain-Barre patients experience their functioning after 1 year.

OBJECTIVE: To analyze how the patient himself perceives his physical and social situation 1 year after Guillain-Barre syndrome (GBS). MATERIAL AND METHOD: The Dutch patients who participated in an international multicenter trial were asked to complete a self-administered questionnaire containing questions on their physical status at homecoming and at 12 months, as well as questions dealing with various aspects of their social condition. RESULTS: Ninety patients participated. Up to 72% had sensory disturbances and loss of power in part of the arms and up to 89% in part of the legs at homecoming. At 12 months, a significant improvement had occurred, but residua were perceived in 36 and 67%, respectively. The residua ranged from irritating to seriously disturbing in up to 49%, and only 33% felt completely cured. Furthermore, 32% had changed their work due to GBS, 30% did not function at home as well as before and 52% had altered their leisure activities. CONCLUSION: One year after the onset of GBS, a considerable number of patients still perceived a decrease of power and sensation with an often disturbing effect. GBS had an evident impact on daily life and social well-being.

Perception of prognostic risk in patients with multiple sclerosis: the relationship with anxiety, depression, and disease-related distress.

OBJECTIVE: The aim of the study was to investigate the impact of perception of prognostic risk on anxiety, depression, and disease-related distress in patients with multiple sclerosis (MS). STUDY DESIGN AND SETTING: Perceived risk and perceived seriousness of the 2-year, 10-year, and lifetime prognosis of wheelchair dependence, disability status, anxiety, depression, and disease-related distress were assessed in 101 patients. Distress was measured as the intrusion and avoidance of MS-related thoughts and feelings. RESULTS: Patients with higher perceptions of 2-year, 10-year, or lifetime risk were bothered by more intrusion of MS-related thoughts and feelings. Only higher perception of the 2-year risk of wheelchair dependence was significantly related with higher levels of anxiety, depression, and avoidance. Similarly, higher perception of the seriousness of wheelchair dependence was consistently associated with more intrusion and avoidance, but only perceived seriousness of the 2-year prospect of wheelchair dependence was significantly correlated with anxiety and depression. All relations were independent of clinically assessed disability status. CONCLUSION: Perceptions of the short-term risk and seriousness of wheelchair dependence were significantly related to anxiety, depression, and disease-related distress in patients with MS. These findings underscore the importance of informing patients with chronic disorders about the short-term prognosis of important long-term consequences of disease.

Comparison between impairment and disability scales in immune-mediated polyneuropathies.

The ability of a scale to detect clinical relevant changes over time, i.e., its "responsiveness," may help clinicians to choose among valid and reliable measures. Therefore, we investigated the responsiveness' rank ordering (best to worse) of six selected valid and reliable scales, namely the Medical Research Council (MRC)-sumscore, sensory-sumscore, grip-strength (Vigorimeter), nine-hole peg, ten-meters walking, and a disability-sumscore, in immune-mediated polyneuropathies. Patients with newly diagnosed Guillain-Barré syndrome (n = 7) or chronic inflammatory demyelinating polyneuropathy (n = 13) were examined over 52 weeks. Responsiveness of each scale was measured using different methods (effect-size, standardized response mean score, Wilcoxon matched-pairs signed-rank, and a newly devised Schmitz's distribution-free responsiveness score), and the obtained scores in each method were plotted against the follow-up period, thus allowing area-under-the-curve calculations (higher area-under-the-curve indicating better responsiveness). Also, longitudinal correlations were performed between the scales' values and patients' own clinical judgments (deteriorated, unchanged, improved) (higher correlation = better responsiveness). A consistent rank ordering was observed in each technique with the disability-sumscore, MRC-sumscore, and Vigorimeter being among the best responsive scales. Hence, the primary use of these measures is suggested in studies of immune-mediated polyneuropathies.

A randomised controlled trial of intravenous immunoglobulin in IgM paraprotein associated demyelinating neuropathy.

This multicentre randomised double blind crossover trial tested the short term efficacy of intravenous immunoglobulin (IVIg) 2.0 g/kg given over 24 or 48 hours in patients with paraproteinaemic demyelinating neuropathy (PDN). Twenty-two patients were randomised and completed the trial. After 2 weeks, the overall disability grade decreased during both IVIg treatment and placebo but neither change was significant nor was the mean difference between the treatment effects. After 4 weeks the overall disability decreased by a mean of 0.55 [0.67] grades during the IVIg period (p = 0.001) while it was substantially unmodified during the placebo period. The mean difference between the treatment effects was significant (p = 0.05). Overall during the IVIg period 10 patients improved and 11 were stable and one got worse. During the placebo period 4 patients improved, 4 deteriorated and 14 were stable. Many secondary outcome measures, including Rankin scale, time to walk 10 metres, grip strength, sensory symptoms score were significantly better during IVIg treatment. Two serious adverse events occurred during the trial, both during placebo treatment. In conclusion the trial showed some short-term benefit of IVIg in about half of the patients confirming previous observation.

Long-term impact on work and private life after Guillain-Barré syndrome.

OBJECTIVE: To determine the long-term impact of Guillain-Barré syndrome (GBS) on work and private life of patients and their partners. METHODS: Three to six years after the onset of GBS 150 patients who participated in the Dutch Guillain-Barré trial received a questionnaire specifically drafted for this study to survey their present psychosocial status. Furthermore, their present physical status was established. RESULTS: A total of 122 patients participated. Thirty-one percent showed moderate to serious physical residua after a functional assessment. Due to GBS, 38% of the patients who held a job had to change it, 44% altered their leisure activities, 37% of the patients did not function as well at home as before GBS and 39% reported a change in their partners' lives. Almost half of the patients still had negative comments on their present psychosocial situation. CONCLUSION: GBS has a serious long-term impact on the patients' work and private life and that of their partners.

Psychometric evaluation of a new handicap scale in immune-mediated polyneuropathies.

A new handicap measure, the Rotterdam nine-item handicap scale, was developed and its validity, reliability, and responsiveness evaluated in patients with immune-mediated polyneuropathies. We evaluated 113 stable patients, of whom 83 had Guillain--Barré syndrome (GBS), 22 had chronic inflammatory demyelinating polyneuropathy (CIDP), and 8 had a gammopathy-related polyneuropathy. We also studied 20 patients with recently diagnosed GBS (n = 7) or CIDP (n = 13) and changing clinical conditions (longitudinal group). Significant discriminatory validity and correlation with the Rankin scale were demonstrated for the Rotterdam nine-item handicap scale (stable group: Spearman's test, r = minus sign.76 to minus sign.78; longitudinal group: intraclass correlation coefficient, r =.83; P <.0001). Also, good reliability (r =.89--.98; P <.0001) and high responsiveness values (standardized response mean values >.8) were obtained for the Rotterdam nine-item handicap scale. In contrast to the Rankin scale, the Rotterdam scale not only provided information regarding mobility but also highlighted physical independence, occupation, and social integration. These results illustrate the clinical usefulness of the Rotterdam nine-item handicap scale under these conditions.