We report an extremely rare case of an endodermal cyst of the cranio-cervical junction located dorsally to the brainstem and upper cervical spine in a 27-year-old female presented with occipital headache, vertigo and pain in both shoulders. Neurological examination showed neck stiffness with bilateral XIth nerve palsy. Magnetic resonance imaging revealed a cystic lesion at the cranio-cervical junction and slight compression of the brain stem. The lesion was totally removed through the posterior approach. The histological diagnosis was endodermal cyst. To our knowledge, the only one such case has been reported in the literature.
Pallister-Hall syndrome (PHS) is extremely rare autosomal dominant disorder with typical clinical features such as presence of polydactyly, hypothalamic hamartoma, bifid epiglottis, anal, renal, genitourinary and pulmonary abnormalities, mainly located in the midline of the body. Spontaneous cerebrospinal fluid (CSF) rhinorrhea is also a rare condition, and it is used to describe nasal discharge of CSF unrelated to previous trauma, localized tumour, surgery, or previous radiation therapy. The exact cause is not yet fully understood.
We report a case of adult female patient previously diagnosed with PHS and late onset of spontaneous nasal liquorrhea, due to defect in the anterior skull base. Although it can be incidental finding, we believe that described defect in the body midline can be another presentation of congenital craniofacial abnormality which are common in PHS.
Dual antiplatelet therapy (clopidogrel and acetylsalicylic acid) is a standard for the embolization of planned intracranial aneurysms with CNS stent due to the possibility of stent thrombus formation. All anti-aggregation drugs, including those listed, have bleeding as a side effect. Three patients with aneurysm had an elevated response to antiplatelet therapy with clopidogrel, which was confirmed by a multiplate test on the "VerifyNow" system. After reducing the dose of clopidogrel or after interrupting it, with the introduction of low molecular weight heparin for the duration of five days, aneurysms were successfully resolved by intracranial implantation of the stent. Perioperative angiograms and postoperative CT angiograms have verified hematomas at the place of punction of the femoral artery. Bleeding was resolved by the femoral artery suture by a vascular surgeon. All patients were discharged home without further complications and with dual antiplatelet therapy. By measuring the platelet function in vitro, the degree of inhibition of platelet activity achieved by the action of the drug can be assessed. A specific test can identify those patients who are highly responsive to the drug with increased platelet reactivity and the possibility of increased risk of bleeding. Our suggestion is to reduce the dosage of clopidogrel or to leave it out for 24 hours with preventive doses of low molecular weight heparin or to change the strategy of treatment of intracranial aneurysm, i.e. avoiding implantation of CNS stent.
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