J. M. Hoff

Pregnancy, delivery, and birth outcome in women with multiple sclerosis

Using data from the compulsory Medical Birth Registry of Norway, the authors investigated the effect of maternal multiple sclerosis (MS) on pregnancy, delivery, and birth outcome in 649 births by MS mothers and 2.1 million control births. The mothers with MS had a higher proportion of neonates small for gestational age and also more frequent induction and operative interventions during delivery.

Myasthenia gravis: Consequences for pregnancy, delivery, and the newborn

Objective: To investigate the effect of maternal myasthenia gravis (MG) on giving birth and on the newborn. Methods: A retrospective cohort study for 1967 through 2000 was undertaken, using data from the Medical Birth Registry of Norway, based on the compulsory notification of all births. The target group consisted of 127 births by mothers with MG. The reference group consisted of all 1.9 million births by mothers without MG. Results: Women with MG had a higher rate of complications at delivery (40.9% vs 32.9%, p = 0.05), and in particular the risk of preterm rupture of amniotic membranes was three times higher in the MG group compared to the reference group (5.5% vs 1.7%, p = 0.001). The rate of interventions during birth was raised (33.9% vs 20.0%, p < 0.001) and cesarean sections doubled (17.3% vs 8.6%, p = 0.001). Five children (3.9%) born by MG mothers had severe anomalies, and three of them died. Conclusions: MG is associated with an increased risk for complications during delivery. This is linked to a higher occurrence of interventions during birth.

Myasthenia gravis in pregnancy and birth: identifying risk factors, optimising care

Women with myasthenia gravis (MG) have increased risk of pregnancy complications and an adverse pregnancy outcome. This study examined risk factors for such complications in order to improve the care for pregnant MG women. Through the Medical Birth Registry of Norway, 73 MG mothers with 135 births were identified. Their obstetrical and clinical records were examined. Data on pregnancy, delivery and the newborn were combined with information on mothers disease. The risk for neonatal MG was halved if the mother was thymectomized (P = 0.03). Children with neonatal MG were more likely to display signs of foetal distress during delivery (P = 0.05). Only in one‐third of the pregnancies did the patient see a neurologist during pregnancy. These patients used MG medication more often during pregnancy (P = 0.001), and were more likely to be thymectomized (P = 0.007). They also had a higher rate of elective sections (P = 0.009). Thymectomy may have a protective effect against neonatal MG. Neonatal MG can cause foetal distress during delivery. Most MG women benefit from being examined by a neurologist during pregnancy, to minimize risks and select the best delivery mode in collaboration with obstetricians.

Myasthenia Gravis: A Review of Available Treatment Approaches

Patients with autoimmune myasthenia gravis (MG) should be further classified before initiating therapy, as treatment response varies for ocular versus generalised, early onset versus late onset, and acetylcholine receptor antibody positive versus MuSK antibody positive disease. Most patients need immunosuppression in addition to symptomatic therapy. Prednisolone and azathioprine represent first choice drugs, whereas several second choice options are recommended and should be considered. Thymectomy should be undertaken in MG with thymoma and in generalised, early-onset MG. For MG crises and other acute exacerbations, intravenous immunoglobulin (IvIg) and plasma exchange are equally effective and safe treatments. Children and females in child bearing age need special attention regarding potential side effects of immunosuppressive therapy. MG pathogenesis is known in detail, but the immune therapy is still surprisingly unspecific, without a pin-pointed attack on the defined disease-inducing antigen-antibody reaction being available.

Pregnancies and deliveries in patients with Charcot-Marie-Tooth disease.

Objective: To investigate the effect of maternal Charcot–Marie–Tooth disease (CMT) on pregnancy and delivery. Methods: Data from the Medical Birth Registry of Norway 1967 to 2002 were surveyed. This registry has compulsory notification of all births. One hundred eight births by mothers with CMT were identified. The reference group consisted of all 2.1 million births by mothers without CMT. Results: Women with CMT had a higher occurrence of presentation anomalies (9.3 vs 4.5%; p = 0.04) and bleeding post partum (12.0 vs 5.8%; p = 0.02). The rate of operative delivery was twice that of the reference group (29.6 vs 15.3%; p = 0.002), and forceps was used three times as often in the CMT group (9.3 vs 2.7; p < 0.001). The majority of CMT cesarean sections were emergency sections. Conclusion: Charcot–Marie–Tooth disease increases the risk for complications during delivery, which is linked to a higher occurrence of emergency interventions during birth.

Arthrogryposis multiplexa congenita: an epidemiologic study of nearly 9 million births in 24 EUROCAT registers.

OBJECTIVE To examine the occurrence of arthrogryposis multiplex congenita (AMC) in Europe and to identify possible risk factors. STUDY DESIGN Retrospective population-based epidemiological study using EUROCAT congenital anomaly registries. The study population included all cases of AMC (based on WHO ICD-9 or ICD-10 codes) that were livebirths (LB), fetal deaths (FD) from 20 weeks gestation and underwent termination of pregnancy for fetal anomaly (TOPFA), 1980-2006. RESULTS Among 8.9 million births covered by 24 EUROCAT congenital anomaly registries, 757 AMC cases were reported. This gives a prevalence of 8.5 per 100,000. Five hundred and four (67%) AMC cases were LB, 199 (26%) cases were TOPFA, and FD occurred in 54 (7%) cases. First week survival status was known for 381 of the 504 LB (76%), of whom 87 (23%) died within the first week of life. Perinatal mortality associated with AMC was 32%. Two hundred and eighty-two (37%) cases had isolated AMC, 90 (12%) had additional syndrome or chromosomal anomalies and 385 (51%) had other major malformations. The same or similar anomaly was reported in 13% of siblings and in 12% of the mothers own family background. Information on prenatal testing was available for 521 cases of which 360 tested positive for a congenital anomaly, representing a sensitivity of 69%. Information on maternal illness before and during pregnancy and medication use in the first trimester was available for approximately a third of the mothers, of whom the vast majority reported no maternal illness or medication use. CONCLUSION AMC is a rare occurrence, with a reported prevalence of 1:12,000. In this study, while information on potential risk factors such as maternal disease or maternal use of drugs was limited, they did not appear to be associated with the occurrence of AMC. AMC was lethal in a third of cases, either in utero or during the first week of life, although this may not be solely attributed to AMC as most cases had additional malformations.

Neurological disorders in the Global Burden of Disease 2010 study

The Global Burden of Disease study (GBD) is a large international initiative to collect and systematize data on disease burden expressed in non‐economic terms, to allow comparisons across different disease conditions and countries.

Cardiovascular and urological dysfunction in spinal cord injury

Hagen EM, Faerestrand S, Hoff JM, Rekand T, Gronning M. Cardiovascular and urological dysfunction in spinal cord injury.
Acta Neurol Scand: 2011: 124 (Suppl. 191): 71–78.
© 2011 John Wiley & Sons A/S.

Artrogryposis multiplex congenita – a rare fetal condition caused by maternal myasthenia gravis

Objectives –  To look at the occurrence of arthrogryposis multiplex congenita in newborn of mothers with myasthenia gravis (MG) and factors connected to this.

Visual impairment in stroke patients – a review

Approximately 30% of all stroke patients suffer from post‐stroke visual impairment. Hemianopia is the most common symptom, but also neglect, diplopia, reduced visual acuity, ptosis, anisocoria, and nystagmus are frequent. Partial or complete recovery of visual disorders can occur, but many patients suffer permanent disability. This disability is often less evident than impairment of motor and speech functions, but is negatively correlated with rehabilitation outcome and can lead to a significant reduction in day‐to‐day functioning. To be visually impaired after stroke reduces quality of life and causes social isolation because of difficulties in navigating/orientating in the surroundings. A thorough diagnosis including targeted examination and later follow‐up with eye examination and perimetry is essential in order to establish the extent of the visual impairment and to select the best rehabilitation strategy. Patients seem to profit from visual rehabilitation focused on coping strategies.