[Cerebral venous thrombosis secondary to essential thrombocythemia]. / Thrombose veineuse cérébrale secondaire à une thrombocytémie essentielle.

INTRODUCTION: Essential thrombocythemia (ET) is a myeloproliferative syndrome; cerebral venous thrombosis (CVT) is a rare complication. OBSERVATION: We report the case of a 20-year-old woman with an uneventful history who was admitted with intracranial hypertension syndrome which had developed over the last four months in association with bilateral decline of visual acuity. Physical examination at admission revealed stage II papilloedema and absence of any focal neurological signs. The brain MRI and the venous MRA showed a thrombosis involving the superior longitudinal sinus and the lateral sinus. The etiological analysis disclosed essential thrombocythemia (ET). The patient was given an antiedema and anticoagulant treatment. Later, an etiological cytoreductive therapy was initiated. Signs of intracranial hypertension regressed progressively with persistence of acute visual disorders associated with sequelar optical atrophy. Discussion. ET is a rare cause of CVT. Reports in the literature have discussed the mechanisms, the physiology, the therapeutic modalities and the clinical course of these CVTs secondarily to ET. CONCLUSION: Stroke, especially transient ischemic attack, is the usual thrombotic expression of myeloproliferative syndrome. CVT is much more exceptional.

[Favorable course of Lance-Adams postanoxic action myoclonus]. / Syndrome de Lance et Adams d'évolution favorable.

Clinical and electrophysiologic data concerning the postanoxic action myoclonus syndrome were described by Lance and Adams in 1963. A patient presented myoclonus involving all parts of the body after laryngospasm. The myoclonus was worsened by emotion and voluntary activity and was clearly attenuated by sleep. Spectacular improvement was observed within one week after valproate and piracetam administration. Clinicians should be aware of this syndrome in order to propose appropriate treatment and avoid delay in the therapeutic decision.

Rigid spine syndrome. Two case-reports.

UNLABELLED: Rigid spine syndrome is characterized by massive spinal rigidity, usually most marked in the cervical region. Stiffness of the peripheral joints is sometimes present. We report two cases. Patient 1 was a 12-year-old boy diagnosed at three years of age with Duchenne's muscular dystrophy because of delayed onset of walking. Contracture of the Achilles tendons, flexion contracture of the elbows, and loss of motion of the cervical spine were the main findings during the current evaluation. Radiographs of the affected joints were normal. An electrocardiogram showed an incomplete left bundle branch block. Muscle enzyme activities were moderately elevated. A myopathic pattern was seen on the electromyogram. A muscle biopsy showed muscle fiber atrophy with peri- and endomysial fibrosis. Patient 2 was a 39-year-old man with a five-year history of isolated rigidity of the cervical spine thought to be due to a spondylarthropathy. Extension was the only movement possible at the cervical spine. The peripheral joints showed no motion range limitation. Findings were normal from radiographs of the spine and sacroiliac joints, an erythrocyte sedimentation rate determination, an electromyogram, and muscle enzyme activity assays. A muscle biopsy showed muscle fiber atrophy with peri- and endomysial fibrosis. DISCUSSION: Rigid spine syndrome is rare in rheumatological practice and can simulate a number of other muscle and joint diseases. Peri- and endomysial fibrosis may be strongly suggestive, although nonpathognomonic. Involvement of the heart governs the prognosis.

Polyradiculoneuropathy revealing a solitary plasmacytoma of the ilium. A new case-report.

Neurological manifestations are uncommon in myeloma patients, and subacute polyradiculoneuropathy as the inaugural manifestations of solitary plasmacytoma of bone is exceedingly rare. We report the case of a 52-year-old man who was evaluated for a three-month history of flaccid tetraplegia with a gradually ascending onset and for a deterioration in general health. Electromyography findings were consistent with polyradiculoneuropathy. Laboratory tests showed a moderate amount of a monoclonal IgG-lambda antibody. Findings were normal from a radiographic bone survey and a radionuclide bone scan. Computed tomography of the pelvis disclosed a solitary osteolytic lesion in the right iliac crest, which was found upon biopsy to be a malignant plasmacytoma. Radiation therapy and chemotherapy were given. Subacute or chronic polyradiculoneuropathy as the inaugural manifestation of solitary plasmacytoma is exceedingly rare and should be distinguished from the sensorimotor polyneuropathy produced by plasma cell infiltration in some multiple myeloma patients. The polyradiculoneuropathy of solitary plasmacytoma can be likened to the neuropathies seen in some forms of multiple myeloma (sclerotic myeloma and POEMS syndrome). The pathophysiology of these neuropathies remains obscure. The case reported here suggests that patients with unexplained lasting polyradiculoneuropathy should be investigated for a plasma cell proliferation even if they have no serum monoclonal component. Because plasmacytomas are painless, imaging studies are needed for their diagnosis. The management of the neuropathy consists in treatment of the tumor.

[Cerebral vascular accidents due to hydatid embolisms. Apropos of 2 cases]. / Accidents vasculaires cérébraux par embolie hydatique. A propos de deux observations.

Hydatid cysts of the brain are very rare. Exceptionally, signs and symptoms are primarily those of acute cerebral ischaemia. Two cases of acute cerebral ischaemia are reported in a 21 year old and 40 year old women. A computed tomographic scan revealed a middle cerebral artery (MCA) infarct and an abrupt cutoff of the MCA at cerebral angiography. A few months later, a CT scan showed cysts in the territory of the infarct. Hydatid cysts were also found in multiple viscera, particularly in the heart. These two observations and some cases reported in the literature suggested that the myocardial cyst may have ruptured into the ventricular cavity, resulting in widespread intravascular dissemination of embryo and causing an acute cerebral infarction. The interest of these cases lies in the rarity of an acute cerebral ischaemia due to hydatid cyst embolism, and in the early diagnosis of cardiac cysts in young patients.