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.

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.

[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.

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.

[2 cases of post-measles myelitis]. / Deux cas de myélite post-rubéolique.

Two cases of a very rare complication of rubella i.e. a strictly spinal cord lesion developing during the course of the viral infection, are reported. In the first case, there was a flaccid paraplegia which incompletely regressed after two months. In the second case there were only sensory disorders and sphincter disturbances and recovery was complete after one month. The post-eruption encephalomyelitis lesions are of uncertain paphogenesis but the most common hypothesis suggested is that of a delayed hypersensitivity mechanism involving a cell-mediated immunologic reaction. The prognosis of these post-injection neurologic manifestations is difficult to assess, but spinal cord lesions could have a good prognosis.

[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.

[Right hemiasomatognosia and sensation of amputation caused by left subcortical lesion. Role of callosal disconnection]. / Hémiasomatognosie droite et sentiment d'amputation par lésion gauche sous-corticale. Rôle de la disconnexion calleuse.

A 72-year-old right-handed hypertensive man presented with a right brachial monoplegia, and hypesthesia of the right half of the body to touch and pricking, sparing the face. A CT scan 2 weeks later showed a spontaneous hyperdense area corresponding to a left subcortical parietal hematoma. The patient used his spontaneous language to express body image disturbances: intense prolonged feeling of amputation related to the upper limb and foot on the right side, hemiasomatognosia without anosognosia, autotopagnosia. Also associated were a right-sided visual negligence and a more general inability to handle spatial data leading to a temporospatial disorientation. Mild language disorders were suggestive of subcortical aphasia: normal incitation and repetition; with semantic paraphasias, poor verbal fluency. Furthermore comprehension of orders or propositions concerning spatial data were poor. Finally, there were signs suggestive of callosal disconnection: paradoxical extinction of the left ear during dichotic listening, agraphia and anomia of the left hand, ideomotor apraxia of the left upper limb, difficulty in visual transfer. The lesion interrupted thalamic tracts to parietal regions and callosal fibers linking parieto-occipital association areas. This twofold lesion was analyzed for each of the neuropsychological disturbances observed. Certain aspect of cerebral function in this patient were reminiscent of "split-brain" disorders. The left hemisphere which "speaks" fails to understand the feelings of the right hemisphere: unfamiliarity of places, sensations of illness, resulting in an unadapted speech. The pathophysiology of feeling of amputation is discussed.

[Isolated sensory deficit of 1 side of the body as a result of a hematoma of the pons]. / Déficit sensitif isolé d'un hémicorps par hématome du pont.

In a patient with a history of sudden onset of an isolated sensory syndrome of the right side of the body the sensory deficit was exclusively related to lemniscal sensitivity. NMR imaging showed a small hemorrhage in the left paramedian portion of the pontine tegmentum, corresponding precisely to the location of the median lemniscus.

[Anterior choroidal artery syndrome. Neuropsychological study of 4 cases]. / Le syndrome de l'artère choroïdienne antérieure. Etude neuropsychologique de 4 cas.

A neuropsychological study was carried out in 4 cases of infarction in the territory of the anterior choroïdal artery. In 3 cases the lesions were on the right side. A syndrome of the minor hemisphere was present with severe visual neglect, constructional apraxia, alexia due to disorders of visuo-spatial strategy, anosognosia and motor impersistence. In the case with a left-sided lesion there was no neglect but a mild aphasia with impaired fluency, semantic paraphasias, perseveration and a decreased psycho-linguistic ability. The mechanisms of neglect in right-sided lesions are considered.

[Tonic deviation of gaze in Wallenberg's syndrome]. / Déviation tonique du regard au cours du syndrome de Wallenberg.

A case of tonic deviation of gaze in a case of Wallenberg's syndrome is presented. Apart from oculostatic signs there were oculokinetic disorders presenting as a dysmetria of saccades with hypometria contralateral to the lesion. The physiopathology of this disorder is discussed. This syndrome of ocular lateropulsion probably results from a lesion of the restiform body interrupting cerebello-vestibular connections, associated with a lesion of the inferior part of the vestibular nuclei.

[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.

[Cavitary orthochromatic leukodystrophy with oligodendroglial changes. A sporadic adult case]. / Leucodystrophie orthochromatique cavitaire avec modifications oligodendrogliales. Un cas sporadique adulte.

A 39 year-old man with mild stable mental retardation, without family history, developed progressively a gait disturbance and intellectual deterioration. CT scan showed a low density of the periventricular hemispheric white matter which increased on subsequent examinations. Eight months before death he presented with several Grand Mal seizures. He died 29 months after the onset of the clinical disorders. Neuropathological studies included light and electron microscopy of a cerebral biopsy and a post-mortem examination of the brain. It showed a sudanophilic leukodystrophy with unusual features: cavitation of the white matter, oligodendrocyte proliferation and lamellar "fingerprint" dense cytoplasmic inclusions in the oligodendrocytes. Only 3 similar cases have been previously reported.

[Anterior choroidal artery syndrome. Clinical and computed tomography study of 4 cases]. / Syndrome de l' artère choroïdienne antérieure. Etude clinique et tomodensitométrique de 4 cas.

The author choroidal artery syndrome is a rare disorder associating, in its complete form, a hemiplegia, a hemianesthesia, and a homonymous lateral hemianopia. Four cases are reported in which the diagnosis was confirmed by a CT scan, which demonstrated a low density area in the posterior part of the posterior limb of the internal capsule, sparing the thalamus medially, and extending laterally to the apex of the pallidum thus corresponding to the area supplied by the anterior choroidal artery. The incomplete forms of this syndrome and clinicopathological correlations are discussed.

[Acquired cerebral toxoplasmosis: 3 anatomoclinical cases]. / Toxoplasmose cérébrale acquise: trois cas anatomocliniques.

Clinical and pathological findings in three adults with toxoplasmosis of the central nervous system are reported. Symptoms and signs in the first patient, a Haitian woman who had lived in France for 2 years, were suggestive of a right hemispheric space-occupying process. The second case was a woman treated for Hodgkin's disease who showed symptoms and signs of a meningoencephalitis. The presenting lesion in case 3 mimicked a tumor of the posterior fossa. Analysis of these 3 cases and of those previously reported underlines: 1) diagnostic difficulties, particularly in immunodepressed patients; 2) the effectiveness of immunoperoxidase for pathological diagnosis; 3) the poor prognosis due to absence of a specific treatment.

[Amyloid neuropathy associated with a benign monoclonal gammopathy (lambda light chains)]. / Neuropathie amyloide associée à une gammapathie monoclonale bénigne (chaines légeres lambda).

The authors report a case of non-familial amyloid neuropathy in which there was sensory-motor neuropathy of the 4 limbs, multiple lesions in the cranial nerves, and large subcutaneous amyloid deposits. Biopsy samples showed the presence of amyloid deposits in the interstitial tissues, the vessel walls in the muscle hypodermis, and in the bone marrow. A light monoclonal lambda chain was present in the serum. Immunofluorescent studies of the biopsy specimens showed the presence of elective fluorescence with an anti-lambda immunoserum in the amyloid substance.

[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.

[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.

[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.