Clinical characteristics and brain PET findings in 3 cases of dissociative amnesia: disproportionate retrograde deficit and posterior middle temporal gyrus hypometabolism.

BACKGROUND: Precipitated by psychological stress, dissociative amnesia occurs in the absence of identifiable brain damage. Its clinical characteristics and functional neural basis are still a matter of controversy. METHODS: In the present paper, we report 3 cases of retrograde autobiographical amnesia, characterized by an acute onset concomitant with emotional/neurological precipitants. We present 2 cases of dissociative amnesia with fugue (cases 1 and 2), and one case of focal dissociative amnesia after a minor head trauma (case 3). The individual case histories and neuropsychological characteristics are reported, as well as the whole-brain voxel-based 18FDG-PET metabolic findings obtained at group-level in comparison to 15 healthy subjects. RESULTS: All patients suffered from autobiographical memory loss, in the absence of structural lesion. They had no significant impairment of anterograde memory or of executive function. Impairment of autobiographical memory was complete for two of the three patients, with loss of personal identity (cases 1 and 2). A clinical recovery was found for the two patients in whom follow-up was available (cases 2 and 3). Voxel-based group analysis highlighted a metabolic impairment of the right posterior middle temporal gyrus. 18FDG-PET was repeated in case 3, and showed a complete functional brain recovery. CONCLUSION: The situation of dissociative amnesia with disproportionate retrograde amnesia is clinically heterogeneous between individuals. Our findings may suggest that impairment of high-level integration of visual and/or emotional information processing involving dysfunction of the right posterior middle temporal gyrus could reduce triggering of multi-modal visual memory traces, thus impeding reactivation of aversive memories.

[Somatoform disorders in neurology visits: history and circumstances: retrospective study of 124 cases]. / Symptômes somatomorphes en consultation de neurologie : expression, soubassement et occasion : étude rétrospective de 124 situations.

We report 124 cases of somatoform disorders, considering psychogenic disorders at the same level as neurological disorders. We noted any psychic, somatic or social condition (history taking) and facilitating circumstances. The patients were aged 16 to 84 years old; 71.7% were women. We observed pain (35.4%), psychogenic headache (25%), sensorimotor loss (27.4%), gait and psychogenic tremor (17.7%), cognitive disorders (11.8%), ocular symptoms (7.2%), and urogenital symptoms (2.4%). Delay to consultation ranged from a few days to 20 years. Psychiatric comorbidity was noted in 30.6% of the cases. In 55.6% of 124 cases, we observed a psychological background. It was a childhood trauma in 15.3% of these cases. In one-third of the 124 situations, we noted an underlying somatic or social condition. Facilitation conditions were frequently mixed. Somatic and/or psychological conditions were noted in one-third of the 124 cases and social conditions in half of them. The neurologist is faced with the challenge of naming the symptom (most often labelled a functional disorder) and of making the decision to stop or limit investigations. Visits by patients with psychogenic disorders make up a significant percentage of neurology speciality appointments. The neurologist should not limit the consultation to differentiating "real" symptoms from psychogenic somatoform disorders, but should also propose a straightforward compassionate approach for effective therapeutic care. By carefully listening to the patient's dialogue, the neurologist can help the patient give meaning to the symptoms, and progress towards improved well-being.

Chronic inflammatory demyelinating polyneuropathy in Waldenström's macroglobulinemia.

INTRODUCTION: Waldenström's disease (WD) is frequently associated with a predominantly sensory neuropathy with a progressive course due to the monoclonal IgM activity against Myelin Associated Glycoprotein (MAG). However, neurolymphomatosis or chronic demyelinating inflammatory polyneuropathy (CDIP) may occur in some patients with WD. CASE REPORT: We report a case of Waldenström's macroglobulinemia in an adult male presenting with cranial nerve palsy and rapidly progressive asymmetric polyneuropathy. Intravenous IgM treatment that provided transient amelioration was followed by a relapse involving tetraparesis. Cerebrospinal fluid analysis, medullar magnetic resonance imaging, and electrophysiological studies led to equivocal findings suggesting the presence of either neurolymphomatosis or CIDP. Finally, sural nerve biopsy results supported the diagnosis of CIDP, which then received appropriate treatment. CONCLUSION: In patients with WD, the possible occurrence of CIDP should be investigated with a neuromuscular biopsy when other investigations are equivocal since the disease calls for a specific treatment.

[Hereditary optic atrophies]. / Les atrophies optiques héréditaires.

INTRODUCTION: Hereditary optic neuropathies, resulting from retinal ganglion cell degeneration, are a heterogeneous group of diseases ranging from asymptomatic forms to legal blindness. STATE OF KNOWLEDGE: Two most frequent phenotypes are Kjer's disease, an autosomal dominant optic atrophy caused by OPA1 gene mutations, and Leber's disease due to maternally inherited mitochondrial DNA mutations. PROSPECTS AND CONCLUSION: Both optic neuropathies usually isolated are sometimes associated with extraocular symptoms, especially neurological symptoms, thus justifying a systematic neurological evaluation and brain imaging.

Contraception and screening for cervical and breast cancer in neuromuscular disease: a retrospective study of 50 patients monitored at a clinical reference centre.

OBJECTIVE: To analyse contraceptive methods and the extent of screening for breast and cervical cancer in women with neuromuscular disease, compare these results with data and guidelines for the general population and determine the environmental and attitudinal barriers encountered. PATIENTS AND METHODS: A retrospective, descriptive study in a population of female neuromuscular disease patients (aged 20 to 74) monitored at a clinical reference centre. RESULTS: Complete datasets were available for 49 patients. Seventy percent used contraception (hormonal contraception in most cases). Sixty-eight percent had undergone screening for cervical cancer at some time in the previous 3 years and 100% of the patients over 50 had undergone a mammography. Architectural accessibility and practical problems were the most common barriers to care and were more frequently encountered by wheelchair-bound, ventilated patients. CONCLUSIONS: In general, the patients had good access to contraceptive care and cervical and breast cancer screening. However, specific measures may be useful for the most severely disabled patients.

An intraventricular clear cell meningioma revealed by an inflammatory syndrome in a male adult: a case report.

Intraventricular meningiomas are infrequent intracranial tumors. Clinical symptoms are mainly due to an increased intracranial pressure or a direct pressure on the surrounding brain structures. Inflammatory syndrome was described in some patients with chordoid meningiomas. Here we report a case of right intraventricular clear cell meningioma in a 50-year-old man who presented with fever, headache, and inflammatory syndrome. Clinical and biological normalization was rapidly obtained after tumor removal. Immunohistochemical examination showed tumor cells and lymphocytes positivity for the pyrogenic cytokine interleukin-6, with a same intensity. To our knowledge, this is the first case described in the literature concerning an adult man with an intraventricular clear cell meningioma associated with a systemic inflammatory syndrome.

An autopsy case of acute multiple sclerosis (Marburg's type) during pregnancy.

We report a case of a 9-month pregnant woman who presented acute psychiatric and neurological symptoms with extensive involvement of the white matter on MRI and no oligoclonal bands on CSF examination. Despite high doses of intravenous steroids, plasmapheresis and immunosuppressive drugs, a fatal outcome (coma) was noted 8 months later. Neuropathological examination confirmed the diagnosis of Marburg's type of multiple sclerosis showing sharp-edged lesions of demyelination, giant astrocytes, numerous macrophages and little perivascular inflammation. We discuss the definition and limits of the Marburg entity with reference to acute disseminated encephalomyelitis, impact of pregnancy, unusual MRI features, neuropathology and treatment.

Cognitive changes in asymptomatic carriers of the Huntington disease mutation gene.

Huntington disease (HD) is a neurodegenerative disorder due to an excessive number of CAG repeats in the IT15 gene on chromosome 4. Studies of cognitive function in asymptomatic gene carriers have yielded contradictory results. This study compared cognitive performance in 44 subjects with the HD mutation (group of carriers) who had no clinical signs of HD and 39 at-risk individuals without HD mutation (group of non-carriers). Neuropsychological evaluation focused on global cognitive efficiency, psychomotor speed, attentional, executive and memory functions. Significant differences, with lower performances in the group of gene carriers, were detected for some measures of psychomotor speed, attention and executive functioning (all P < 0.01). More differences between groups were observed for memory measures, in particular on the California Verbal Memory Test. Complementing these observations, cognitive scores were correlated with age in the group of gene carriers, but not in the group of non-carriers. This suggests that the cognitive changes precede the appearance of the motor and psychiatric symptoms in HD and that tests proved to be sensitive to early HD deficiencies are better suited than global cognitive efficiency scales to observe them.

[Two consecutive episodes of intracerebral hemorrhage as the presenting feature of polyarteritis nodosa]. / Deux hémorragies cérébrales révélatrices d'une périartérite noueuse.

We report a case of polyarteritis nodosa revealed by intracranial haemorrhage. A 40-year-old woman presented two episodes of cerebral haemorrhage twelve days apart, the second due to an aneurysm rupture. The diagnosis of polyarteritis nodosa (PAN) was based on the following criteria: histological aneurysm examination, angiography suggesting PAN with cerebral, renal and splenic localizations, loss of weight and cutaneous nodules. Cerebral haemorrhage in PAN is rare and exceptionally the presenting feature of the disease.

Fibromuscular dysplasia may herald symptomatic recurrence of cervical artery dissection.

BACKGROUND: The prevalence of fibromuscular dysplasia (FMD) in patients with cervical artery dissection (CAD) is unknown. Our objectives were to assess the risk of CAD recurring as a stroke or a transient ischemic attack and the association of these events with FMD. METHODS: We prospectively included and followed 103 consecutive patients who had been admitted for a CAD. The median follow-up was 4 years (range 4 months to 10 years). The main criteria for inclusion were a mural hematoma demonstrated by cervical magnetic resonance imaging and/or signs suggesting CAD on 2 other investigations. FMD was diagnosed on the so-called string of beads pattern by digital subtraction angiography. RESULTS: Five patients had CAD recurrence (60% occurred late). Four of these 5 patients had FMD. In 4 patients, CAD recurrence involved another cervical artery. CONCLUSION: The rate of symptomatic CAD recurrence was 1% per year and was often related to FMD.

[A study of action planning in patients with Alzheimer's disease using the zoo map test]. / Etude de la planification de l'action au moyen du test du plan du zoo dans la maladie d'Alzheimer.

INTRODUCTION: Executive dysfunction is regularly reported in patients with Alzheimer's disease. Nevertheless few studies have focused on planning ability in this neurodegenerative disease. OBJECTIVES: This study aimed to investigate the formulation and the execution of plans in Alzheimer's disease using an ecological planning subtask derived from the Behavioural Assessment of the Dysexecutive Syndrome test battery, the "Zoo Map Test". There are two trials. The first trial consists of a "high demand" version of the subtask in which the subjects must plan in advance the order in which they will visit designated locations in a zoo (formulation level). In the second, or "low demand" version, the subject is simply required to follow a concrete externally imposed strategy to reach the locations to visit (execution level). The test was given to 16 patients with Alzheimer's disease and 13 normal elderly subjects. RESULTS: The two way ANOVAs mainly showed more difficulties in patients with Alzheimer's disease than in healthy elderly in both conditions. The difference between formulation and execution was greater in patients with Alzheimer's disease than in healthy elderly. Planning impairments mainly correlated with behavioural changes (in particular motivational changes) observed by patient's relatives. CONCLUSION: These results suggest that patients with Alzheimer's disease have some problems to mentally develop logical strategies and to execute complex predetermined plans, which are partially related to behavioural changes.

[Demyelinating neuropathy during anti-TNF alpha treatment with a review of the literature]. / Neuropathie démyélinisante au cours d'un traitement par anti-TNF alpha et revue de la littérature.

INTRODUCTION: Tumor necrosis factor- (TNF) blockers are efficient in the treatment of autoimmune disorders such as inflammatory bowel disease and rheumatoid arthritis, but can induce CNS adverse effects including retrobulbar optic neuritis or aggravation of multiple sclerosis. OBSERVATION: We report a case of progressive demyelinating polyneuropathy after initiation of Adalimumab (Humira). Corticosteroid and intravenous immunoglobulins were ineffective but the neuropathy improved within six months after adalimunab discontinuation. DISCUSSION: This case, and other reports recently published suggest that anti-TNF alpha drugs can induce demyelinating neuropathy. CONCLUSION: Clinicians should be on the lookout for signs evocating neuropathy in patients given anti TNF alpha.

[Idiopathic acute transverse myelitis: application of new diagnosis criteria to 17 patients]. / Myélite aiguë transverse idiopathique: application des nouveaux critères diagnostiques à une cohorte de 17 patients.

INTRODUCTION: Idiopathic Acute Transverse Myelitis (ATM) is an inflammatory and immune-mediated disorder, distinct from infectious ATM, ATM of systemic lupus erythematosus or Sjögren's syndrome, and medullary manifestation of multiple sclerosis. Prognosis is not well-known. OBJECTIVE: To evaluate clinical, paraclinical and pronognosis data in patients selected with new diagnosis criteria, classically described in idiopathic ATM. METHODS: Seventeen patients with diagnosis criteria were retrospectively (1996-2005) studied. A telephone investigation was conducted in 2005 to obtained data on the clinical course. RESULTS: Seven men and 10 women, ranging in age from 15 to 75 years (mean: 39.8 years) met these new criteria. Our study showed that epidemiological and clinical findings as well as laboratory results were in agreement with those presented in the literature. Conversely, prognosis was better since 76p.cent of the patients could walk without assistance. The clinical presentation of some of our patients and/or their progression towards other multifocal inflammatory disorders, suggests there might be links between ATM, neuromyelitis optica (NMO) and Acute Dissemined Encephalomyelitis (ADEM). CONCLUSION: Patients with idiopathic ATM, selected with new criteria, have a rather good prognosis. ATM seems to be part of a continuum of neuroimmunologic disorders including NMO or ADEM although reasons explaining distinct focal disorders remain unclear.

Psychiatric side effects of interferon-beta in multiple sclerosis.

Psychiatric disorders, especially depression, are frequent in patients with multiple sclerosis (MS). They are attributed both to the psychosocial impact of a chronic, usually progressive, disabling illness and to cerebral demyelination. Besides, drugs such as corticosteroids and possibly interferon (IFN) may also have depressogenic effects. Major depressive disorders and/or suicidal ideation are a major concern and efforts to identify and minimize these reactions are of much importance. Psychiatric side effects, particularly depression, are widely reported with IFN-alpha and have been suspected with IFN-beta but are not yet fully established. Our review of the literature revealed that most studies discard an association between IFN-beta and depression or suicide. However, few patients, especially those with a history of depression, might be at higher risk for depression when treated with IFN-beta. Overall, considering the uncertainty of a link between IFN-beta and depression and/or suicide, as well as the complete remission of psychiatric complications after IFN discontinuation and/or antidepressant treatment, physicians should closely monitor the psychiatric status of patients, but should not refrain from including them in IFN-beta treatment programs, even when they have past or present depression.

Idiopathic acute transverse myelitis: application of the recent diagnostic criteria.

Despite an extensive diagnostic workup, some cases of acute transverse myelitis (ATM) remain of unknown etiology and have been referred to as "idiopathic" by the Transverse Myelitis Consortium group. In a retrospective study of 288 patients with ATM, 45 cases (15.6%) met the criteria for idiopathic ATM. The patients formed a relatively homogeneous group in terms of clinical and MRI data, but the prognosis was highly variable.