[Intramedullary tuberculoma: a case report]. / Tuberculome intramédullaire. Un cas.

A fifty-year-old Portuguese man presented with a six-month history of low back pain, which initially was mechanical and slowly became inflammatory. Secondarily, he complained of right atypical sciatalgia. He did not report any fever, loss of weight, cough nor personal or familial history of tuberculosis. General examination was normal. Neurologic examination showed weakness of the extensors of the right leg, with a symmetric increased reflexes of the lower extremities suggesting a pyramidal syndrome without Babinski's sign. Laboratory data were normal as well as chest radiographs. Dorsolumbar gadolinium enhanced MRI revealed an intramedullary ringlike enhancing mass at T12 level. Lumbar puncture showed 11 WBC/mm3 (95 p. 100 lymphocytes), a normal protein and glucose content. PCR and culture for Mycobacterium tuberculosis were negative. Within a few days, he developed meningoencephalitis with fever, CSF examination revealed then 360 WBC/mm3 (65 p. 100 lymphocytes and 17 p. 100 neutrophils), a protein content of 7 g/l and a glucose level of 1.7 mmol/l. The clinical picture was then suggestive of tuberculosis and a specific therapy with rifampin, izoniazid, pyrazinamid, ethambutol and steroids was started. Clinical improvement and a second CSF culture that revealed one month later Mycobacterium tuberculosis complex confirmed this diagnosis. Ten months later, the patient was asymptomatic with a normal MRI. To our knowledge, this is the first total recovery of an intramedullary tuberculoma on medical therapy alone, confirmed by MRI normalization. We reviewed also 19 recent cases of tuberculomas in the literature, intending a therapeutic attitude when discovering an intramedullary ringlike enhancing mass on MRI.

[Leber "plus" disease: optic neuropathy, parkinsonian syndrome and supranuclear ophthalmoplegia]. / Maladie de Leber "plus": neuropathie optique, syndrome parkinsonien et ophtalmoplégie supranucléaire.

A 34-year-old right handed man presented with a bilateral subacute optical neuropathy associated with cervical dystonia, parkinsonism and supranuclear ophtalmoplegia. Magnetic resonance imaging showed in T2 increased intensity of signal in the dorsal mesencephalum and pons as well as in dorsal part of striata. The 3,460 mutation of mitochondrial DNA was found in a blood sample. This observation adds to the variability of presentation of Leber's "plus".

Comparison between oral and written spelling in Alzheimer's disease.

Written and oral spelling were compared in 33 patients with Alzheimer's disease (AD) and 25 control subjects. AD patients had poorer spelling results which were influenced by orthographic difficulty and word frequency, but not by grammatical word class. Lexical spelling was also more deteriorated than phonological spelling. Moreover, oral spelling was more impaired than written spelling in AD patients, whereas no difference was present between oral and written spelling of controls. Analysis of spelling errors showed that, for controls, errors were predominantly phonologically accurate in both spelling tasks. Significantly, AD patients produced more phonologically accurate than inaccurate errors in written spelling, whereas these errors did not differ in oral spelling. In contrast to controls who produced more constant than variable responses in oral and written spelling, AD patients made more variable responses (words correctly spelled in one task but incorrectly in the other) and they showed many instances of variable errors (different misspellings from one spelling task to the other). Two stepwise regression procedures showed that written misspellings were specifically correlated with language impairment, whereas oral spelling errors were correlated with attentional and language disorders. These results suggest that AD increases the attentional demands of oral spelling process as compared to written spelling. This dissociation argues, either for a unique Graphemic Buffer in which oral spelling requires more attentional resources than written spelling or for the hypothesis of separate buffers for oral and written spelling.

Risk of cancer from azathioprine therapy in multiple sclerosis: a case-control study.

An increased risk of cancer has been reported in patients treated with azathioprine. To assess the long-term risk of neoplasia in azathioprine-treated multiple sclerosis (MS) patients, we conducted a case-control study using the Lyon Multiple Sclerosis Database. From the 1,191 MS patients included in the database, we identified patients who developed cancer before December 31, 1991. Each case was then matched to three cancer-free MS controls by gender, date of birth, and date of MS onset. A matched analysis was performed to compare cases and controls for exposure to azathioprine therapy during the same follow-up period. Twenty-three MS patients with cancer were identified: 17 solid tumors, 2 skin carcinomas, 4 hematopoietic cancers. Cases had a mean age of 34.5 years +/- 10.2 (+/- SD) at clinical onset of MS and have been followed up for an average 13.8 years +/- 8.1 before being diagnosed with cancer. Fourteen cases (61%) and 34 controls (49%) had been treated with azathioprine for at least 1 month after being diagnosed with MS (adjusted odds ratio = 1.7; 95% confidence interval [CI], 0.6 to 4.6). When assessing risk associated with different durations of azathioprine therapy compared with no treatment at all, we found that MS patients had an increase in cancer risk of 1.3 (95% CI, 0.4 to 4.0) when treated less than 5 years, of 2.0 (95% CI, 0.4 to 9.1) when treated 5 to 10 years, and of 4.4 (95% CI 0.9 to 20.9) when treated more than 10 years. Similar results were obtained when assessing cancer risk associated with cumulative doses of azathioprine ever taken. This case-control study suggests that the overall long-term risk of cancer from azathioprine is low in MS patients. The results are suggestive of a dose-response relationship with no significant risk during the first years of treatment and a possible increased risk after about 10 years of continuous therapy. Further studies are needed to better assess the risk-benefit ratio of azathioprine in MS.

Clinical and radiological aspects of Villaret's syndrome.

A 68-year-old man with a history of large cell lung carcinoma presented 1 year after surgical management of the initial lesion, with a complete unilateral IX-XII cranial nerve palsy with Horner's sign. This rare multiple cranial nerve palsy is called Villaret's syndrome. It suggests an extracranial lesion located in the retroparotid space. Complete basal skull radiology work up including computed tomography and magnetic resonance imaging confirmed the location of the causal lesion in the retroparotid space.

Comparative study of oral and written picture description in patients with Alzheimer's disease.

Oral and written picture descriptions were compared in 22 patients with Alzheimer's disease (AD) and 24 healthy elderly subjects. AD patients had a significant reduction of all word categories, which, similarly to controls, was more pronounced in written than in oral texts. They also reported fewer information units than controls, but without task difference. At the syntactic level, written descriptions of AD subjects were characterized by a diminution of subordinate clauses and a reduction of functors. More grammatical errors were present in written descriptions by AD and control subjects. AD and control groups produced an equivalent number of semantic errors in both tasks. However, in oral description, AD patients had more word-finding difficulties. In sum, AD descriptions were always shorter and less informative than control texts. Additionally, written descriptions of AD patients appeared shorter and more syntactically simplified than, but as informative as oral descriptions. Whereas no phonemic paraphasias were observed in either group, AD patients produced many more graphemic paragraphias than controls produced. Furthermore, written descriptions had more irrelevant semantic intrusions. Thus, as compared to oral descriptions, written texts appeared to be a more reliable test of semantic and linguistics difficulties in AD.

[Multiple (visceral and neurologic) tumors]. / Les tumeurs multiples (viscérales et neurologiques).

OBJECTIVES: We studied the frequency, histology, epidemiological features and delay between tumours with one localization in the central nervous system. We searched for an association between neurological and visceral neoplasia and tried to establish a guideline when central nervous system tumours appear after a visceral tumour. METHODS: From January 1973 to December 1993, we archived all patients admitted for neurological tumours or who had tumours discovered in our ward. Both neurological and non-neurological and malignant and non-malignant tumours which appeared simultaneously or after a delay were recorded. We retained only patients who had one neurological and one or more visceral tumours (n = 11). Multiple neurological or visceral tumours and phacomatosis were excluded. RESULTS: The association between neurological and visceral tumours is an uncommon but not exceptional finding as multiple tumours were seen in 1% of the cases admitted during the study period with a neurological or a visceral tumour. Meningioma and glioma were the most frequent histological type of central nervous system tumours (10 cases out of 11). Malignant melanoma was particularly frequent among the systemic tumours (3 out of 13 tumours). The classical association between meningioma and malignant breast neoplasia was absent in our study but an association of malignant melanoma and tumours of the central nervous system was found in 3 patients. Age and sex were similar for neurological and visceral tumours. The delay separating the discovery of the two tumours was shorter when the first tumour was a neurological tumour. CONCLUSIONS: These findings demonstrate the frequency of associated neoplasia. We did not find the classical association between breast cancer and meningioma but did find one between malignant melanoma and tumours of the central nervous system. This short study emphasizes the usefulness of having histological proof (when possible) before considering a cerebral tumour as a metastasis of a visceral tumour.

[Progressive lupus dementia. 2 cases with or without antiphospholipid antibodies]. / Démence lupique progressive. Deux cas avec anticorps antiphospholipides ou non.

Two cases of lupus dementia presented many points of particular interest: 1) the progressive installation of intellectual deterioration, inaugural for the first observation; 2) the diagnostic difficulties of neurolupus with the ARA criteria; 3) the appearance of cerebral magnetic resonance imaging with confluent hypersignals of the periventricular white matter on T2-weighted images; 4) the patholophysiological hypotheses: vascular disease? immunologic disease?; 5) the clinical improvement and SPECT amelioration for the second patient with corticosteroids.

[False negative results by X scanners in intracranial tumors in adults]. / Les faux négatifs du scanner X en pathologie tumorale intracrânienne de l'adulte.

We report a cohort of 34 cases, collected between 1978 and 1992, presenting with symptomatic intracranial tumours with a first negative CT scan. Subsequently, the tumours was revealed with a second CT scan or with an another neuroradiological technique (MRI or angiography). These "false negatives" represented 5% of all intracranial tumours seen during the same period of time in the same institution. Gliomas were observed in 67.7% of cases (a majority of them with a high grade). Initially, seizure was the most common clinical presentation (61.8%). Mean delay between the first scan and the definitive diagnosis was 13.4 months. The localization was supra-tentorial in 79.4% and infra-tentorial in 20.6%. This study leads us to discuss the attitude when a first CT scan is normal, more particularly in cases presenting with seizures, and the different factors that can explain the normality of this first CT scan.

[An aspect of intracerebral lymphoma in scanner X mimicking meningioma]. / Aspect d'un lymphome intracérébral au scanner X suggérant un méningiome.

We report a case of primary non hodgkin's lymphoma of the central nervous system mimicking a meningioma on CT scan. We discuss the main characteristics of the diagnosis of this tumor, particularly based on the radiological presentation.

[Hemiballismus with logorrhea and thymo-affective disinhibition caused by hematoma of the left subthalamic nucleus]. / Hémiballisme avec libération thymo-affective et logorrhée par hématome du noyau sous-thalamique gauche.

A 71 year-old hypertensive right-handed man presented a small intracerebral haematoma limited to the left subthalamic nucleus that resulted in right hemiballismus and a neurobehavioral disinhibition syndrome. The latter associated logorrhoea and euphoria, but there was no frontal signs nor mental confusion. The patient kept full insight into his mental change, that contrasted with his prior personality. Hemiballismus and the manic-like state resolved within 2 months. This case and some others from the literature illustrated that a focal lesion in the basal ganglia could result in manic-like affective change, and that a relation did exist between hyperkinetic syndrome and euphoria. Our case contrasted with most of the published cases of neurobevioral changes following basal ganglia discrete lesions, expressing as hypokinesia, apathy and loss of motivation. With reference to the functional circuits linking the frontal cortex and the basal ganglia, as described by Alexander, this syndrome of combined disinhibition of motricity, speech and affect could involve either the lateral orbito-frontal or the anterior cingulate loops, more especially the indirect pathways travelling through the subthalamic nucleus, lesion of which is known to disinhibit thalamo-cortical activity.

Striatocapsular infarction: MRI and MR angiography.

We present a case of left striatocapsular infarction manifest clinically as a transient right hemiparesis. MRI showed a left striatocapsular infarct. Striatocapsular infarction, unlike lacunar infarction, is often associated with occlusive disease of the carotid artery. In order to screen the carotid vessels, cervical MR angiography (MRA) was performed during the same examination, demonstrating a left internal carotid artery occlusion, confirmed by contrast arteriography. MRA, a noninvasive modality, can be a useful adjunct to MRI, when diagnostic information concerning the cervical carotid artery is needed.

Congenital protein C deficiency and superior sagittal sinus thrombosis causing isolated intracranial hypertension.

The first case of a superior sagittal sinus thrombosis causing isolated intracranial hypertension as a result of congenital protein C deficiency is reported. Such a possibility must not be overlooked. Anticoagulation is recommended as a treatment for cerebral venous thrombosis. In the case of congenital protein C deficiency, vitamin K antagonists must be started cautiously due to the risk of skin necrosis.

Idiopathic intracranial hypertension after ofloxacin treatment.

We report the occurrence of idiopathic intracranial hypertension in a patient treated with ofloxacin, a fluoroquinolone antimicrobial agent, for 16 months. The withdrawal of ofloxacin and acetazolamide therapy were followed by a complete recovery of visual function.

[Arnold-Chiari malformation in adults. Development of diagnostic conditions since the availability of MRI]. / Malformation de Chiari de l'adulte. Evolution des conditions diagnostiques depuis l'IRM.

We reviewed retrospectively 47 patients with a Chiari malformation (CM) who had been admitted to the Clinique de Neurologie of Lyon from 1971 to 1991. The purpose of this report is to assess the possible influence on the frequency and the clinical pattern at presentation of CM since 1985 when Magnetic Resonance Imaging (MRI) became available to us. A diagnosis of CM was made for 21 patients before 1985 (group 1) and for 26 patients thereafter (group 2), i.e., a 2.5 increase of incidence of CM frequency (1.5 patients/year before 1985 vs 3.7 patients/year thereafter). This increase was even more striking for cases without syringomyelic symptoms and signs (SSS) (42.9% in group 1, vs 76.9% in group 2). The accuracy of clinical diagnosis was 68.1% for the total surveyed period (90.5% before 1985 and 50% thereafter). For cases with SS at presentation, a syringomyelic cavity was radiologically demonstrated in 80% of the cases in the pre-MRI period and 90.9% of the cases since MRI was available. On the other hand, when a syringomyelic cavity was radiologically demonstrated, there was a related clinical SS in 70% of group 1 patients and 50% of group 2 patients.

[Association of amyotrophic lateral sclerosis and multiple sclerosis]. / Association sclérose latérale amyotrophique et sclérose en plaques.

A twenty-five year old woman developed a progressive right hemiparesis which remitted within three months, without treatment. The diagnosis was a first relapse of multiple sclerosis. After a 10 year fully asymptomatic period, the patient developed weakness of the legs with falls and swallowing difficulties. Fasciculations and amyotrophy were present in the limbs and the tongue. There were no sensory abnormalities. The electromyogram confirmed the peripheral neurogenic degeneration with signs of anterior horn involvement. Motor and sensory nerve conductions were normal. Muscle weakness and atrophy increased in the limbs and the bulbar territory and the patient died nine months later. The autopsy showed characteristic "old" plaques of multiple sclerosis in the cerebrum with anterior horn cell and pyramidal tracts degeneration, typical of amyotrophic lateral sclerosis, in the spinal cord. Although exceptional, this association of amyotrophic lateral sclerosis and multiple sclerosis leads to the discussion of an etiological immunological dysregulation common to these two diseases.

[Calcified cerebellar metastasis of carcinoma of the uterine cervix]. / Métastase cérébelleuse calcifiée d'un carcinome du col utérin.

A case of calcified cerebellar metastasis from a squamous cell carcinoma of the uterine cervix is reported. The literature on the rare occurrence of both intracranial metastasis from uterine cervix carcinoma and calcifications in intracranial metastasis is reviewed.