Spontaneous intracranial haemorrhage is one of the cerebrovascular complications in beta-thalassaemia major patients. This is a report of 2 cases of fatal intracranial haemorrhage. Their ages were 12 and 7 years respectively, and they had been receiving regular blood transfusion for the past 3 and 2 years respectively. They developed acute onset of headache, loss of consciousness and convulsions at 5 and 2 days respectively after their last blood transfusion. C-T scan of the brain showed massive intracranial haemorrhage with extension into the ventricles. The spontaneous intracranial haemorrhages in these two cases was probably multi-factorial in origin. The predisposing factors included recent blood transfusion, prolonged prothrombin time and partial thromboplastin time as well as reduced platelet count.
A rare syndrome of hypertension, seizures and intracranial bleed has been reported among patients with congenital hemolytic anemia who underwent multiple blood transfusions. We report this syndrome in a 12-year-old Malay girl with hemoglobin E-beta-thalassemia, who underwent intensive transfusion and subsequently had headache, visual loss, severe hypertension and seizures. A comprehensive literature review revealed 30 patients with this syndrome, of whom 15 had intracranial bleed and 12 among these 15 died. A less-intensive transfusion regimen among patients with chronic hemolytic anemia and prompt detection and management of hypertension may prevent this potentially fatal syndrome.
The outcome of 109 patients with severe head injury was studied in relation to clinical and computed tomographic (CT) criteria on admission, after resuscitation. Age, Glasgow Coma Score (GCS) and state of pupils strongly correlated with outcome. The presence of hypothalamic disturbances, hypoxia and hypotension were associated with an adverse outcome. The CT indicators associated with poor outcome were perimesencephalic cistern (PMC) obliteration, subarachnoid haemorrhage, diffuse axonal injury and acute subdural haematoma. The prognostic value of midline shift and mass effect were influenced by concomitant presence of diffuse brain injury. For the subset of patients aged < 20 years, with GCS 6-8 and patent PMC (n = 21), 71.4% correct predictions were made for a good outcome. For the subset of patients aged > 20 years, with GCS 3-5 and partial or complete obliteration of PMC (n = 28), 89.3% correct predictions were made for a poor outcome.
Intracranial haemorrhage is a major cause of severe morbidity and mortality in child abuse cases in developed countries. However, similar data are not available in most developing countries. This study therefore aimed to determine the incidence of intracranial haemorrhage amongst all cases of child physical abuse, the nature of the injuries incurred, and the morbidity and mortality resulting therefrom. Among 369 cases of physical abuse seen over a 4-year period, 41 (11.4%) had intracranial haemorrhage, of whom 37 (90%) were 2 years old or less. A history of trauma was present in only eight (20%), of which only two were compatible with the injuries incurred. Subdural haemorrhages accounted for 80% of the cases, with skull fractures present in only nine cases. Fifty-four per cent of the 37 children aged 2 years of age or less had no external signs of trauma, but 11 of them had retinal haemorrhages. This is in contrast to the children older than 2 years of age who all had external signs of trauma. The overall prognosis was dismal with an early mortality of almost 30% (13 cases) and at least seven cases with severe neurological sequelae. These findings are comparable with studies from developed countries which have established that non-accidental injury must be considered as a cause of intracranial haemorrhage in any young child, despite the absence of external signs of trauma.
Thirteen cases of late haemorrhagic disease of infancy due to vitamin K deficiency presenting with intracranial haemorrhage were seen over a three - year period from 1984 to 1986. The clinical picture was fairly typical; a short history of being unwell (poor feeding, vomiting, irritability, high pitched cry, fits) and physical findings of pallor, a normal body temperature, impairment of consciousness, abnormal respiration and a very tense anterior fontanelle. Vitamin K deficiency was implicated by the prolonged prothrombin time which rapidly returned to normal with vitamin K injection. The outcome was poor. Possible factors giving rise to vitamin K deficiency are discussed. The author suggests the introduction of the giving of vitamin K to all new-borns.
A case of an intracerebral bleed in a young man with a rare combination of arteriovenous malformation (AVM) and unilateral moyamoya disease is presented. The location of the bleed in the left basal ganglia corresponded to the area supplied by the basal moyamoya vessels. The AVM which received supply from collateral moyamoya vessels as well as normal cerebral arteries was located in the ipsilateral parieto-occipital region posterior to the basal ganglia bleed. This is the first reported cerebral AVM co-existing with a unilateral moyamoya disease in the English literature. Unusual features of the case such as the unilaterality of the angiographic abnormalities, their coexistence and hypotheses as to their development are discussed.
Stroke is one of the leading causes of death worldwide, and spontaneous bleeding into the brain parenchyma, intracerebral haemorrhage (ICH), is a stroke subtype associated with high morbidity and mortality. Overall, it comprises about 15% of all stroke in Caucasians, this figure being much higher in Asians and black people. Blood pressure (BP) appears to play an important role in this disease. We have reviewed available literature on the relationship of BP to the occurrence of primary and secondary ICH, the association of BP levels measured early after stroke with prognosis and complications, and evidence about the effects of early BP lowering treatments on post-stroke outcomes. BP appears to be an important risk factor for primary and secondary ICH. In addition, high BP early after ICH may be detrimental to outcome, possibly contributing to complications such as rebleeding and haematoma enlargement. Few data are available about the effects of early lowering of BP on outcome after ICH with no reliable trial yet conducted. Proper randomised trials are required to establish the effect of early lowering of BP on outcome after ICH.
Retinal changes are common in adult acute leukaemia patients at presentation, but whether they correlate with the risk of subsequent intracranial haemorrhage is unknown. A 4-year study has been carried out in 82 newly-diagnosed acute leukaemia patients, aged 12-77 years, who were studied prospectively for the presence of intra-retinal haemorrhages (IRH), white-centred haemorrhages (WCH), cotton-wool spots (CWS) and macular haemorrhages (MH). Groups with and without these features were compared for their risk of intra-cranial haemorrhage (ICH) within the first 30 d following diagnosis. There was no association between the incidence of ICH and the presence of IRH, WCH or CWS. However, 6/13 of those with MH developed ICH, compared to 6/69 of those without MH (relative risk 5.0, CI 95% [2.03-12.33], P=0.003). The only other identifiable risk factor for ICH was the M3 subtype of AML, but if the four cases of M3-AML were discounted from analysis, MH remained a highly significant risk factor for ICH. Patients with MH should be monitored intensively for the development of ICH, and receive priority in the allocation of platelets where these are in short supply.
Dexamethasone has recently been shown to block the production of cachectin (implicated in the pathogenesis of cerebral malaria) if administered prior to endotoxin induction of mouse macrophages. Using the hamster cheek pouch-cerebral malaria model, we tested the hypothesis that dexamethasone is effective as a therapeutic agent in severe malaria if given before some yet undefined trigger point in the disease. Infected hamsters were treated with dexamethasone (0.7 mg/kg) daily on days 7-12, 4-12, or 1-12 post-challenge. When treatment was started on day 1, whole body oxygen consumption (used as a measure of erythrocyte transport to sites of diffusion) on day 12 was greater than (P less than 0.05) that of infected control animals, though the degree of anemia was no different in treated and untreated groups. Furthermore, treatment produced a reduction in monocyte accumulation, capillary malfunction, and monocyte/red blood cell aggregate formation observable in the cheek pouch in vivo and a similar reduction in monocyte presence, capillary pathologic change, and multifocal hemorrhage in the brain on postmortem. These data suggest that mediator(s), whose production can be blocked by pretreatment with dexamethasone, are involved in the pathogenesis of disease leading to death of the Plasmodium berghei infected hamster.
We report a newborn infant girl, born to consanguineous parents, with recurrent intracranial hemorrhage secondary to congenital factor V deficiency with factor V inhibitor. Repeated transfusions of fresh-frozen plasma (FFP) and platelet concentrates, administrations of immunosuppressive therapy (prednisolone and cyclophosphamide), and intravenous immunoglobulin failed to normalize the coagulation profiles. Exchange transfusion followed-up by administrations of activated prothrombin complex and transfusions of FFP and platelet concentrates caused a temporary normalization of coagulation profile, enabling an insertion of ventriculoperitoneal (VP) shunt for progressive hydrocephalus. The treatment was complicated by thrombosis of left brachial artery and ischemia of left middle finger. The child finally died from another episode of intracranial hemorrhage 10 days after insertion of the VP shunt.