Bilateral cystic encephalomalacia following multiple intrauterine transfusions for anti-Kell isoimmunisation.

Fetal and neonatal haemolytic diseases result from maternal allo-immunisation to fetal antigens. Maternal antibodies cross the placenta causing red cell haemolysis, resulting in fetal anaemia and, in severe cases, hydrops and perinatal death. Intravascular intrauterine blood transfusion (IUT) has markedly reduced perinatal mortality and is now a standard procedure. IUT is considered to be a safe procedure with fetal loss rate reported to be less than 5% and no reported increase in the rate of neurodevelopment impairment. In this report, we are presenting a case of bilateral cystic encephalomalacia following fetal anaemia secondary to anti-Kell iso-immunisation treated with multiple IUTs. Such a significant adverse outcome following IUT for anti-Kell iso-immunisation has not been reported in the literature. This case highlights the need for appropriate parental counselling and routine postnatal head ultrasound in all babies delivered following multiple IUTs.

Cerebrospinal fluid oligoclonal IgG bands in patients with spinal arteriovenous malformation and structural central nervous system lesions.

OBJECTIVE: To investigate the incidence and characteristics of patients with structural central nervous system (CNS) lesions and cerebrospinal fluid oligoclonal IgG bands. DESIGN: A retrospective study. METHOD: The medical records of patients with cerebrospinal fluid oligoclonal IgG bands were evaluated for the presence of structural CNS lesions, their location and cause, and for clinical characteristics. SETTING: Cerebrospinal fluid oligoclonal IgG bands were examined in the Neuroimmunology Laboratory, Hadassah University Hospital, Jerusalem, Israel. PATIENTS: Two hundred seventy of 570 patients with positive cerebrospinal fluid oligoclonal IgG bands were available for analysis. Twenty patients had structural CNS lesions. RESULTS: Twenty (7.5%) of the 270 patients had structural CNS lesions: 3 patients had spinal arteriovenous malformation; 5 patients had tumors; 9 patients had compressive cervical myelopathy. Traumatic leukomalacia, Arnold-Chiari malformation type 1, and CNS hemosiderosis were present in 1 patient each. In 2 patients (1 patient with recurrent meningioma and 1 patient with posttraumatic encephalomalacia) the presence of a structural CNS lesion was followed by the development of multiple sclerosis. In all 3 patients with spinal arteriovenous malformation, oligoclonal IgG identification prolonged the time to diagnosis and therapy, which varied from a few weeks to 3 years. CONCLUSIONS: Structural CNS lesions, responsible for the neurological disorder, were present in 20 patients (7.5%) with cerebrospinal fluid oligoclonal IgG bands. The mechanism underlying oligoclonal IgG presence in spinal arteriovenous malformation and the coexistence of multiple sclerosis and structural CNS lesions is unknown, but may be related to recurrent tissue damage with repeated presentation of CNS antigens to the immune system.

Immunohistological study of encephalomalacia in pigs infected with Aujeszky's disease virus.

Seven hysterectomy-derived colostrum-deprived pigs aged 4 weeks were inoculated intranasally with 10(3) plaque-forming units (1 ml) of the Yamagata YS-81 strain of Aujeszky's disease virus. One pig died and five developed encephalomyelitis and trigeminal ganglionitis. Three pigs killed on days 12-16 showed prominent malacic degeneration. Associated with the malacic foci were many lysosome-positive cells. IgG- and IgM-containing cells in the perivascular cuffs and glial nodules were first detected on day 7, after which they increased in number. They were thought to be closely associated with the presence of neutralizing antibody. These findings suggest that inflammatory cells in the brain are of haematogenous origin.

Cystic brain lesion in utero.

Two autopsy cases of cystic brain lesion in utero are reported. One of them was a donor infant of twin transfusion syndrome. The baby died immediately after birth and showed multicystic encephalomalacia in the distribution of the anterior cerebral artery. The second baby was a stillborn infant with thanatophoric dwarfism with associated chronic periventricular leukomalacia (PVL). It was suggested that the multicystic encephalomalacia and chronic PVL found in the first and second cases were caused by persistent circulatory disturbances in utero.