[Neurosyphilis revealed by a multiple cranial neuropathy: magnetic resonance imaging findings]. / Neurosyphilis révélée par une atteinte multiple des nerfs crâniens: apport de l'imagerie par résonance magnétique nucléaire.

INTRODUCTION: We report the magnetic resonance imaging (MRI) findings in a case of neurosyphilis revealed by the involvement of two cranial nerves. CASE REPORT: A 41-year-old man developed a right cochleovestibular and left trigeminal neuropathy, associated with high serum titers of VDRL and TPHA, high titers of TPHA in the cerebrospinal fluid (CSF) and several CSF oligoclonal IgG bands. On MRI, hypertrophy and gadolinium contrast enhancement of these cranial nerves were associated with several supratentorial cortical nodules surrounded by marked cerebral edema, corresponding to syphilitic gummas. One of these cortical nodules was biopsied. Microscopic examination showed lesions of meningoencephalitis with necrosis and granulomatous vasculitis. After penicillin therapy, the serum VDRL titers and the MRI abnormalities disappeared, a partial clinical recovery was observed and a significant reduction of the serum TPHA titers was found. DISCUSSION: Such MRI abnormalities are not specific and can be observed in various tumoral, auto-immune and infectious diseases. They can also mimic neurofibromatosis type II. Cranial nerve involvements in neurosyphilis can result from nerve inflammation in basal meningitis, nerve ischemia in meningovasculitis or from compression by an adjacent gumma. In our case, the cranial neuropathy was related to a mixed meningovascular and parenchymatous form of neurosyphilis.

Mitoxantrone-related acute leukemia in two MS patients.

We report two new cases of mitoxantrone-related leukemia occurring in two patients with multiple sclerosis (MS), 14 and 18 months after the last infusion of the drug. One patient was successfully treated. We were able to collect 29 other cases in the literature. Most of them were single reports but some were described within cohorts of mitoxantrone-treated MS patients. The incidence rate was 0.65% from all cohorts totalizing 2299 patients. Acute promyelocytic leukemia with the translocation t(15;17) was over-represented in the MS population in comparison with cancer patients also treated with mitoxanrone. The occurrence of leukemia was dose-independent and appeared with a mean delay of 20 months after the end of the treatment.

[Chorea-athetosis in the anti-Hu syndrome]. / Mouvements choréo-athétosiques et syndrome anti-Hu.

INTRODUCTION: Paraneoplastic choreo-athetoses are rare. We report a case of anti-Hu syndrome with choreo-athetosis. CASE REPORT: A 48-year-old woman developed a small-cell lung carcinoma revealed by an anti-Hu syndrome. The neurological features included choreo-athetosis predominating in the upper limbs, chronic sensorimotor axonal polyneuropathy, and opsoclonus. The cerebrospinal fluid was acellular and contained several oligoclonal IgG bands, not found in the corresponding serum. Magnetic resonance imaging revealed bilateral high-intensity lesions on T2/FLAIR sequence in the corona radiata. Moderate transitory improvement of the paraneoplastic neurological syndrome was observed after several carboplatin-etoposid cycles. CONCLUSION: A paraneoplastic origin must be considered in all cases of unexplained choreo-athetosis. Paraneoplastic choreo-athetosis is most often associated with other neurological symptoms. The most frequent associated tumor is a small-cell lung carcinoma with anti-CRMP5 and/or anti-Hu antibodies. Our patient developed paraneoplastic choreo-athetosis related to an anti-Hu syndrome in the absence of anti-CRMP5/CV2 antibodies. Paraneoplastic choreo-athetosis might result from a central lesion, and/or from proprioceptive deafferentation subsequent to peripheral neuropathy.

[Thoracic myelopathy revealing a chronic ossificans arachnoiditis of the thoraco-lumbar spinal cord]. / Myélopathie dorsale révélant une arachnoïdite spinale chronique dorso-lombaire ossifiante

We report a case of chronic spinal, ossificans, thoraco-lumbar arachnoiditis, associated with an arachnoidal cyst at the level of TH7, and revealed by a rapidly progressive thoracic myelopathy. Two years ago, he had presented with a spinal intrathecal haemorrhage of unknown etiology. There was no evidence of spinal traumatism, arterial hypertension or coagulation disorder. The finding of several small cysts and angiomas in the liver of this patient lead us to suspect an associated spinal vascular malformation, not detected on the spinal magnetic resonance imaging. A partial neurologic improvement was observed after laminectomy and cysto-peritoneal derivation.

MRI of the spine in cobalamin deficiency: the value of examining both spinal cord and bone marrow.

We observed a case of pernicious anaemia in which MRI of the spine demonstrated both intrinsic lesions of the spinal cord and abnormal signal in the bone marrow. The latter resolved with replacement therapy. Only partial recovery of the cord lesions was observed.

[Kufs disease with leukoencephalopathy]. / Maladie de Kufs avec leucoencéphalopathie.

We report a case of adult neuronal ceroid lipofuscinosis (Kufs' disease) with leukoencephalopathy on cerebral scan CT and MRI. A 52 year-old woman presented with partial complex epileptic seizure followed by progressive dementia, cerebellar ataxia, pyramidal and akineto-rigid signs and symptoms. After 6 years of evolution, cerebral stereotactic biopsies showed a diffuse gliosis of the white matter, but no clear demyelination. Nerve and glial cells contained numerous PAS+ autofluorescent granules. In the oligodendrocytes and astrocytes of the white matter these granules appeared electronmicroscopically as cytoplasmic osmiophilic lamellar bodies with fingerprint profile combined with some curvilinear and rectilinear aspects. The cortical nerve cells contained granular osmiophilic bodies. This "leukoencephalopathic" variant of Kufs' disease is probably related to the pigmentary type of orthochromatic leukodystrophy, wherein similar inclusions have been only described in the macrophages and glial cells of the white matter.

[Cerebral cysticercosis treated by albendazole: development of cerebral magnetic resonance imaging]. / Cysticercose cérébrale traitée par albendazole: évolution de l'imagerie par résonance magnétique cérébrale.

A 36-year-old Indian man presented with a generalized tonic-clonic seizure. The diagnosis of neurocysticercosis was suspected by the presence of multiple cystic and nodular cerebral lesions on MRI, and was confirmed by serological studies (ELISA). He was treated with albendazole during one week at the daily dose of 15 mg/kg. This case illustrates the advantage of MRI over CT for the detection and the therapeutical follow-up of neurocysticercosis. Moreover, the MRI findings can be correlated to the anatomical stage of neurocysticercosis, proposed by Escobar (1983); four stages are described: I = vesicular stage (living larvae), II = vesicular colloidal stage (degenerating larvae), III and IV, granular nodular and nodular calcified inactive stages respectively.

[Agenesis of the corpus callosum, heterotopia of the gray cortex and interhemispheric cyst. Late radiologic diagnosis in an asymptomatic adult]. / Agénésie calleuse, hétérotopie de substance grise et kyste interhémisphérique. Diagnostic radiologique tardif chez un adulte asymptomatique.

Agenesis of the corpus callosum may occur as an isolated finding; most often, it is present in association with other cerebral malformations such as cortical heterotopias. Computerized tomography and magnetic resonance imaging are methods of choice to detect these cerebral prenatal abnormalities, especially asymptomatic forms. We report the case of an asymptomatic young adult with complete callosal agenesis, cortical heterotopia and interhemispheric cyst, on CT and MR imaging.