A case series of hemorrhagic neurological complications of sickle cell disease

Abstract

Sickle cell disease (SCD) is a group of hemoglobinopathies that vary in severity, the most severe form, homozygous sickle cell anemia, is more commonly associated with neurologic complications. These are attributed to the vaso-occlusion and micro-obstruction in the circulation of the central nervous system. The incidence of various neurologic complications in SCD ranges from 6% to 30% in various series. The commonly reported in literature include silent cerebral infarction (SCI), ischemic stroke, transient ischemic attacks (TIAs), headaches, seizures and neurocognitive impairment. However, hemorrhagic complications like subarachnoid hemorrhage (SAH), hemorrhagic stroke, extradural and subdural hematomas, especially in absence of trauma are rarely thought of. We report three uncommon spontaneous hemorrhagic manifestations of sickle cell anemia – one case of parenchymal (intracerebral) bleed who presented with acute onset of parkinsonism and two cases of extradural hematoma (EDH) of which one patient had recurrent EDH at the same site which is hitherto not reported in the literature.

Massive spontaneous subdural hemorrhage mimicking dural venous thrombosis in a sickle cell adolescent, a rare case report

“…This implies to both cases of acute or chronic subdural hemorrhage, initial evaluation of the patient's condition may determine the choice of management [ 16 ]. In case of subdural hemorrhage which are asymptomatic are managed by observation mainly by medical management including intracranial pressure control using drugs like mannitol, seizure prophylaxis, and reversal of anticoagulation where necessary [ 5 ]. Surgery is indicated in case of neurological deterioration of the patient such as a low Glasgow coma score, evidence of mass effect with cerebral compression on computed tomography such as collection thickness greater than 10 mm.…”

“…Tanzania alone accounts for 11,000 sickle cell disease births annually [ 4 ] making it one of the commonest hemoglobinopathy in this country. Sickle cell disease as most of its complications causes vaso-occlusion and microvascular obstruction of small peripheral blood vessels such as those supplying the brain [ 5 ] that eventually leads to devastating neurovascular complications such as cerebral infarction and ischemic stroke which is reported to be higher [ 5 ]. Hemorrhagic complications of sickle cell such as subdural, epidural, subarachnoid hemorrhages or intra-cerebral in the absence of trauma have been rarely observed [ 6 ].…”

Acute soft head syndrome in a teenager with sickle cell anemia: A case report

“…14 Thirdly, repeated VOCs could lead to multiple, but subtle microinfarction, which over time results in altered bone and periosteal structures, bone thinning and local vessel wall necrosis leading to non-traumatic blood extravasation to subgaleal and epidural spaces. 11,15 The diagnosis of ASHS is largely clinical. However, imaging using computed tomography (CT) scan is prudent which typically shows evidence for EMH as demonstrated by diffuse widening of the diploic space in the skull and thinning of the inner cortex due to infarction.…”

“…Secondly, hypoxia triggers angiogenic responses, which results in the formation of fragile local vascular beds, which act in tandem, under the context of increased cardiac output to precipitate bone breakage and extravasation of blood into the subgaleal space 14 . Thirdly, repeated VOCs could lead to multiple, but subtle microinfarction, which over time results in altered bone and periosteal structures, bone thinning and local vessel wall necrosis leading to non‐traumatic blood extravasation to subgaleal and epidural spaces 11,15 …”

“… 14 Thirdly, repeated VOCs could lead to multiple, but subtle microinfarction, which over time results in altered bone and periosteal structures, bone thinning and local vessel wall necrosis leading to non‐traumatic blood extravasation to subgaleal and epidural spaces. 11 , 15 …”