Neurosurgical aspects of dialysis-related spinal amyloidosis: Report of three cases and a review of the literature.

BACKGROUND AND PURPOSE: Osteoarticular manifestations of beta-2 microglobulin amyloidosis are often diagnosed in long-term dialyzed patients. However, spinal involvement is rare (10-25% of patients), and generally not associated with neurological deterioration. Compression of the spinal cord or roots is extremely rare, and probably under-recognized. METHODS: The authors describe three cases of spinal stenosis presenting with neurological signs in long-term dialyzed patients, prospectively collected over 2 years in two different institutions and treated by surgical decompression. In all three cases, the main cause of neural compression was amyloid deposition in the spine, either extradurally in the ligamentum flavum or intradurally. RESULTS: All patients improved after surgery and did not present any postoperative complications. However, two out of three patients with amyloid in the cervical spine required surgical revision to obtain a satisfactory decompression of the spinal cord. DISCUSSION: The authors discuss spinal amyloidosis which is a well-known complication of long-term dialysis. However, neurological complications such as spinal cord or radicular symptoms have been rarely reported and, when present in dialyzed patients, are symptoms that are often attributed to other causes. To our knowledge, this is the first case series that demonstrates the relationship between neurological deterioration and amyloid depositions in the spinal canal that occur in long-term dialyzed patients. The prevalence of spinal stenosis related to the presence of amyloid in this specific subgroup of patients is probably underestimated.

Posterior reversible encephalopathy syndrome (PRES) and hypomagnesemia: A frequent association?

Posterior reversible encephalopathy syndrome (PRES) is a serious neurological condition encountered in various medical fields. Pathophysiological factor(s) common to PRES cases of apparently unrelated etiologies are yet to be found. Based on the hypothesis that hypomagnesemia might participate in the cascade leading to PRES, our study sought to verify whether hypomagnesemia is frequently associated with PRES regardless of etiology. From a retrospective study of a cohort of 57 patients presenting with PRES of different etiologies, presented here are the findings of 19 patients with available serum magnesium levels (SMLs) during PRES. In the acute phase of PRES, hypomagnesemia was present in all 19 patients in spite of differences in etiology (including immunosuppressive drugs, hypertensive encephalopathy, eclampsia, systemic lupus erythematosus, iatrogenic etiology and unknown). SMLs were within normal ranges prior to PRES and below normal ranges during the first 48h of PRES, with a significant decrease in SMLs during the acute phase. In this retrospective study, constant hypomagnesemia was observed during the acute phase of PRES regardless of its etiology. These results now require larger studies to assess the particular importance of acute hypomagnesemia in PRES and especially the possible need to treat PRES with magnesium sulfate.

[Pupil-sparing painful oculomotor neuropathy due to a carotico-cavernous aneurysm]. / Ophtalmoplégie extrinsèque pure douloureuse du nerf moteur oculaire commun révélant un anévrysme de la carotide.

INTRODUCTION: In third cranial nerve palsy, the lack of mydriasis evokes a noncompressive mechanism. Case report. We report a case of a slightly painful, pure extrinsic third-nerve palsy, complete except for the partial ptosis secondary to the compression by an intracavernous carotid aneurysm. Percutaneous endovascular embolization was followed by complete regression of the palsy within 4 weeks. The pupil may have been spared by the mechanism of compression after the separation of the pupillomotor and extrinsic fibers or the ischemia of the third nerve by its arterial trunk lesion. CONCLUSION: This case report underlines that brain radiological explorations are necessary in some third-nerve pupil-sparing palsies.

[Crowned dens syndrome: three new cases]. / Syndrome de la dent couronnée : à propos de trois nouveaux cas.

INTRODUCTION: Crowned dens syndrome is due to a microcrystalline infringement (hydroxyapatite or calcium pyrophosphate) of the retro-odontoidal ligament of atlas, often leading to the erroneous diagnosis of meningitis or spondylitis. We report on three new cases diagnosed from 1996 to 1999. EXEGESIS: The patients complained of cervicalgies, headaches or fever. The initially evoked diagnoses were meningitis, spondylodiscitis or endocarditis. Clinical exam found meningism and an inflammatory syndrome in all patients. Analysis of the cerebro-spinal fluid realised in two cases was normal. The diagnosis of crowned dens syndrome was assessed in two cases by cervical CT scan of C1/C2. In the third case, chondrocalcinosis of a wrist allowed this diagnosis. We report a probably non fortuitous case of crowned dens syndrome associated with genetic hemochromatosis. A non steroidal anti-inflammatory treatment allowed a dramatic regression of clinical symptoms. CONCLUSION: This entity should be better known; it can mimick numerous diagnosis and be responsible for fever in the long course.

Thromboembolic events after endovascular treatment of unruptured intracranial aneurysms in two patients with antiphospholipid-antibody syndrome.

Antiphospholipid antibodies (APAs) are circulating immunoglobulins associated with a hypercoagulable state. The antiphospholipid syndrome combines APAs and clinical manifestations, including arterial or venous thromboses and/or recurrent spontaneous fetal loss. The main risk incurred by endovascular treatment of intracranial aneurysms is the occurrence of thromboembolic events. We report two cases of patients with antiphospholipid syndrome who developed thromboembolic complications after the endovascular treatment of unruptured intracranial aneurysms.

Neuroimaging features of spontaneous intracranial hypotension.

We reviewed the cranial MRI and radionuclide cisternograms of four adults with postural headache indicating spontaneous intracranial hypotension (SIH). All four underwent clinical and radiological follow-up. MRI showed diffuse, thin meningeal enhancement; bilateral subdural fluid collections; and morphological abnormalities secondary to "sagging" of the brain. Radionuclide cisternography revealed direct or indirect signs of leakage of cerebrospinal fluid (CSF) along the spinal axis, and the symptoms resolved after the leak treated by epidural injection of blood at a level indicated by the cisternogram. The diffuse meningeal enhancement decreased but persisted on follow-up MRI, although the patients were asymptomatic. All morphologic abnormalities resolved during 3-5 months follow-up.

Intracranial aneurysm associated with relapsing polychondritis.

We describe a 50-year-old man with relapsing polychondritis (RP) involving auricular cartilage, uveitis and hearing loss, who had an aneurysm of the anterior cerebral artery. Intracranial aneurysm is a rare manifestation of RP.

Radionuclide cisternography in spontaneous intracranial hypotension.

We report a case of spontaneous intracranial hypotension (SIH) that was investigated using cranial MRI and radionuclide cisternography. Radionuclide imaging was remarkable, showing direct signs of diffuse asymmetric leakage and indirect signs of cerebrospinal fluid (CSF) hypotension consisting of slow CSF circulation to the convexity and rapid appearance of urinary bladder activity. The MRI appearance was also suggestive of SIH, with diffuse meningeal enhancement. Treatment with autologous blood injection at the level of the radionuclide spinal leakage was useful, resulting in disappearance of SIH symptoms.

[Antiphospholipid syndrome. 20 cases]. / Syndrome des antiphospholipides. 20 observations.

OBJECTIVES: We analyzed the clinical and biological characteristics as well as the clinical course and outcome observed in 20 patients with antiphospholipid antibodies and clinical signs including thrombosis or repeated spontaneous abortion to better identify the recently described antiphospholipid syndrome. METHODS: We retrospectively studied all patients observed in our unit from 1981 to 1992 who fulfilled the following inclusion criteria: a) at least one episode of arterial or venous thrombosis and/or repeated spontaneous abortions, b) positive for antiphospholipid antibodies. RESULTS: Twenty patients were included, 3 with systemic lupus erythematosus (according to the American Rheumatism Association criteria). Arterial or venous thrombosis occurred in 9 and 16 respectively, including exceptional cases of cerebral phlebitis and thrombosis of dermal capillaries. High blood pressure was recorded in 8. Only 1 or 2 types of antiphospholipid antibodies were found in most patients. Anticardiolipin, a circulating anticoagulant and a false-positive Bordet-Wassermann reaction were found together in only 3 out of 16. In addition, the antibody level varied independently from the thrombotic events. There was no case with a clinical course from primary antiphospholipid syndrome to systemic erythromatosus lupus. The effect to treatment on occurrence of new thrombotic events was studied. Three patients suffered one or more haemorrhagic events during antivitamin K treatment. CONCLUSION: It is difficult to establish a differentiation between primary antiphospholipid syndrome, systemic lupus erythematosus and lupus-like syndromes, and precise methods of identifying antiphospholipid antibodies should be further developed.

[Does arthritis due to varicella zoster virus exist?]. / L'oligo-arthrite zostérienne existe-t-elle?

Occurrence of monoarthritis or oligoarthritis during the course of zoster is exceedingly rare (three previously reported cases). In the additional case reported herein, an immunocompetent female exhibited oligoarthritis (right shoulder followed by two metacarpophalangeal joints in her right hand) a few days before developing a typical zoster eruption in the ipsilateral upper limb. Pathogenesis of zoster-related arthritis is discussed. The most likely hypothesis is infection of the joint via the nerve supply rather than via the bloodstream. Evidence in support of this mechanism includes recovery of the virus from synovial fluid specimens in a previously published case.

[Hypothyroid myopathy: apropos of a case with a great increase of creatine phosphokinase, myoglobinemia and transient kidney failure]. / Myopathie hypothyroïdienne: à propos d'un cas avec élévation majeure de la créatine phosphokinase, myoglobinémie et insuffisance rénale transitoire.

Clinical, usually moderate myopathy, frequently develops in patients with hypothyroidism and may reveal the thyroid dysfunction. Biological signs of myolysis, essentially a, usually moderate, rise in creatine phosphokinase plasma levels, are often found. On the other hand, rhabdomyolysis seems to be exceptional in this context. We report a case where an extremely severe clinical and biological myopathy associated with transient renal failure revealed hypothyroidism due to prolonged intake of amiodarone.

[Cerebral infarct and pure sensory deficit]. / Infarctus cérébraux et déficit sensitif pur.

Twenty cases of pure sensory deficit of vascular origin are reported in patients aged 36 to 79 years. This type of attack in usually presumed to be due to a thalamic lacuna in the ventro-postero-lateral nucleus. However, other reported cases have shown other causal mechanisms and lesional sites. In the present series, CT scan and MRI in 11 cases demonstrated: infarctions in 9 and hemorrhages in 2 patients. The ventro-postero-lateral nucleus was involved in only 4 cases. An infarct of the posterior limb of the internal capsule (anterior choroidal artery territory) was present in 4 cases. The lesions in the other patients were: a thalamic hemorrhage and a small pontine hemorrhage lesion. The likely cause was embolism of cardiac origin in 4 cases, hypertension in 11 cases and diabetes in 4. One patient had an aneurysm of the posterior cerebral artery.

Subcortical neglect.

The unilateral neglect syndrome is defined as a lack of directed attention to one half of the space or the body. It has been reported mainly after cortical lesions of the right parietal or frontal lobe in man. However, there is strong experimental and clinical evidence for the existence of neglect syndromes of subcortical origin. The unilateral neglect syndrome is not the result of elementary sensory deficits. The failure in directed attention is dependent on an imbalance of hemispheric activation.

Infarction in the territory of the anterior choroidal artery. A clinical and computerized tomographic study of 16 cases.

Sixteen cases of the anterior choroidal artery syndrome are reported. In its completed form, this rare syndrome combines the triad of hemiplegia, hemianaesthesia, and homonymous hemianopia. CT examination confirms the diagnosis by revealing an area of reduced density situated in the posterior limb of the internal capsule, sparing the thalamus medially and encroaching upon the tip of the globus pallidus laterally, and corresponding to the distribution of the anterior choroidal artery. Incomplete forms of the syndrome are more frequent. Left-sided spatial neglect may accompany right-sided lesions, as may slight disorders of speech in left-sided lesions. Clinical-anatomical correlations are discussed.

[Thalamic neglect]. / Négligence thalamique.

Thalamic lesions can provoke negligence behavior with various, sometimes dissociated manifestations: motor negligence or reduction in use of one half of the body whatever the direction of the gesture or the half-space in which it occurs; extinction phenomena demonstrated without difficulty for visual, somatesthetic and auditory modalities; spatial negligence involving attentional and intentional components, often difficult to distinguish. Negligence of this type is mainly the result of right sided lesions, with several non-exclusive explanations: natural predominance of the left hemisphere, activating role of language with respect to the left hemisphere, capacity of the right hemisphere to be "attentive" to bath half-spaces in contrast to the left hemisphere, attentive to the right half-space only.