Moschcowitz syndrome mimicking basilar pathology

Abstract

Background: Thrombotic thrombocytopenic purpura (TTP) is a clinically heterogeneous syndrome first described by Moschkowitz in 1924. Often postinfectious, but also autoimmune- or drug-associated, TTP is characterised by thrombocytopenia, Coombs-negative haemolytic anaemia, neurological deficits, renal impairment and fever. Inhibitory antibodies against von Willebrand factor (vWf)-cleaving protease lead to a functional deficiency of this protease and to unusually large multimers of vWf. These multimers are capable of agglutinating circulating platelets, thereby reducing platelet count and causing thrombotic microangiopathy concomitantly. Patients: A 68 year old female patient (A) was transferred to our neurological intensive care unit with an acute loss of consciousness and unprotected airways following a four days' history of dysarthria, vertigo and slow decline of the level of consciousness. Eight months later, a 31 year old woman (B) was admitted to our unit with an acute loss of consciousness and bilateral up going plantars, after she had been treated for headache and paraesthesias of her right face and upper limb for seven days. Examinations: On admission, patient A had intermittent right-sided gaze palsy and bilateral up going plantars. Patient B showed a left-sided gaze and head turn palsy plus a left-sided hemiparesis. Basilar thrombosis was ruled out by CT-angiography (A), and by contrast enhanced MR-angiography (B). MRI-scans showed ischaemic lesions bilaterally in the MCA-territory (A), and in the right thalamus (B). Laboratory testing revealed haemolytic anaemia and thrombocytopenia in both cases. The fraction of fragmentocytes in the differential blood count was highly increased. Treatment and outcome: Plasma exchange against FFP was commenced on the admission day in both cases. It was continued until the platelet count had stabilized and the fraction of fragmentocytes had decreased. Patient A was extubated after two days of treatment and could be transferred with a slight dysarthria and dysphagia. Patient B intermittently developed a left-hemispheric syndrome, but slowly improved under treatment. She was discharged with a mild psychosis. Conclusions: We present two cases with severe CNS manifestation of a TTP. All patients with unexplained neurological symptoms, thrombocytopenia and haemolytic anaemia should immediately be transferred to a specialist unit.