[Severe PERM syndrom mimicking tetanus]. / PERM syndrome sévère mimant un tétanos.

We report the case of a 55-year-old man without significant medical history admitted to the ICU for a progressive paralysis mimicking life-threatening tetanus. Evolution with classical tetanus treatment was negative, with the need for ventilator support and worsening condition being life threatening. Uncommon evolution revealed a rare glycin antibody-associated hyperekplexia (progressive encephalomyelitis with rigidity syndrome). Patient dramatically improved with immunosuppressive therapy including plasmatic exchanges, cyclophasmid and high dose corticoid infusions. Intensivists should be aware of this very rare syndrome whose treatment is the opposite of tetanus while presentation is very close. Optimal and treatment could lead to prolonged survival.

Limbic encephalitis and type 1 diabetes with glutamic acid decarboxylase 65 (GAD65) autoimmunity: improvement with high-dose intravenous immunoglobulin therapy.

Glutamic acid decarboxylase antibodies (GAD-abs) are an immunological factor involved in type 1 diabetes and other diseases involving the central nervous system. This report is of a patient with type 1 diabetes and a rare case of non-paraneoplastic limbic encephalitis mediated by anti-GAD65 antibodies that improved with the use of immunosuppressive drugs.

[Paraneoplastic neurological syndromes]. / Les syndromes neurologiques paranéoplasiques.

Paraneoplastic neurological syndromes (PNS) are uncommon and defined by an acute or subacute neurological syndrome associated with a cancer. These syndromes often antedate the diagnostis of the underlying neoplasia that is usually not clinically evident. An early diagnosis is the main condition for a better neurological and carcinologic outcome. Subacute cerebellar ataxia, subacute sensitive neuropathy, limbic encephalomyelitis, Lambert-Eaton myasthenic syndrome, opsoclonus myoclonus, dermatopolymyositis and intestinal pseudo-obstruction belong to the well-characterized PNS and their presence must lead to onconeuronal antibodies (ONA) detection. Treatment of the underlying neoplasia is the mainstay of treatment to obtain a better outcome. However, recent findings lead to consider immunological specific treatment according to the subtype of associated ONA. PNS associated with ONA targeting membrane antigens are thus usually responsive to immunomodulator therapies. Conversely, PNS associated with ONA targeting intracellular antigens are of poor outcome despite mild improvement under immunosuppressive therapies in some patients.

Comorbidity between temporal lobe epilepsy and depression: a [18F]MPPF PET study.

Brain and brainstem changes of serotoninergic 5-hydroxytryptophan (5-HT)(1A) receptor density have been reported in patients with major depressive disorder as well as in patients with temporal lobe epilepsy (TLE), using PET and the selective antagonist radiotracers [(11)C]WAY-100635 or [(18)F]FC-WAY. We used a distinct 5-HT(1A) antagonist, [(18)F]MPPF, whose binding potential depends on both receptor density and extracellular serotonin concentration, in 24 patients with drug-resistant TLE and MRI evidence of hippocampal sclerosis but without prior antidepressant exposure. Their Beck Depression Inventory (BDI-2) score ranged from 0 to 34, with nine patients having a score >11. We used a simplified reference tissue model, statistical parametric mapping and anatomical regions of interest (ROIs) to correlate parametric images of [(18)F]MPPF BP with the total BDI score and its four subclasses. The total BDI score, as well as symptoms of psychomotor anhedonia and negative cognition, correlated positively with [(18)F]MPPF BP in the raphe nuclei and in the insula contralateral to seizure onset, whereas somatic symptoms correlated positively with [(18)F]MPPF binding potential in the hippocampal/parahippocampal region ipsilateral to seizure onset, the left mid-cingulate gyrus and the inferior dorsolateral frontal cortex, bilaterally. We confirm an association of depressive symptoms in TLE patients with changes of the central serotoninergic pathways, in particular within the raphe nuclei, insula, cingulate gyrus and epileptogenic hippocampus. These changes are likely to reflect lower extracellular serotonin concentration in more depressed patients, with an upregulation of receptors a less likely alternative.

[Syndrome of headache with neurologic deficits and CSF lymphocytosis]. / Syndrome de céphalées et déficits neurologiques transitoires avec lymphocytose du liquide céphalorachidien.

INTRODUCTION: A syndrome of headache with neurologic deficits and cerebrospinal fluid (CSF) lymphocytosis is uncommon and clinicians should be aware of this entity. EXEGESIS: We report a 28-year-old man without previous medical history of migraine, who presented with severe headache and temporary focal, neurological deficits. Lumbar puncture revealed aseptic lymphocytic pleiocytosis. The patient completely recovered within one month. This condition was suggestive of a transient syndrome of headache with neurologic deficits and lymphocytosis. The main characteristics and the pathophysiology of this uncommon disorder, generally with a benign course, are discussed. CONCLUSION: Such syndrome of headache, neurologic deficits and CSF lymphocytosis should be included in the differential diagnosis of meningo-encephalitis. The constant benign course of this affection should be emphasized.

Concomitant bone marrow metastasis of a glioblastoma multiforme revealed at the diagnosis.

The rare occurrence of extra-neural metastases in patients having a tumour of the central nervous system (CNS) could mean that the symptoms of a metastatic lesion are confused with a second pathology. We recently treated a patient with a glioblastoma multiforme who was developing a pancytopaenia at the initial diagnosis. The frequent red cell and platelet transfusions were transitorily active. An extensive radiological investigation and a unilateral iliac bone marrow aspirate and biopsy were performed. Cells immunoreactive to glial fibrillary acidic protein were detected in a specimen obtained from the iliac bone. Post-mortem examination confirmed metastasis to extra-cranial bone and revealed other metastases in lung, mediastinal lymph node and spleen. Therefore, in patients with malignant glioma tumours, bone marrow metastasis, though not common, should be investigated when bone pain or cytopaenia occur.

[MRI findings in a case of prolonged status epilepticus]. / Suivi iconographique d'un etat de mal epileptique prolonge.

We report the MR imaging findings in a 20 year old woman with status epilepticus of more than 3 months duration following an episode of lymphocytic meningitis. Repeated MR examinations showed progressive symmetrical cortical lesions, followed by subcortical and basal ganglia lesions which evolved to cortical laminar necrosis and hemorrhagic necrosis with eventual subcortical cerebral atrophy. This case has similarities with animal status epilepticus models. Biological investigations were all negative. This suggests that the brain lesions may be related to the prolonged status epilepticus.