Eric Mormont

Atopic myelitis: a clinical, biological, radiological and histopathological diagnosis.

We describe a young patient with an unusual intramedullary lesion filled with eosinophils. The 21-year-old man developed chronic myelitis without optic neuritis or signs of systemic or infectious disease. A spinal biopsy was conducted because of the progressive extension and pseudo-tumoural aspect of the lesion. Histopathological analysis of the biopsy specimen revealed a severe inflammatory process with macrophages and numerous eosinophils. The eosinophil count in the blood and cerebrospinal fluid (CSF) were normal. Clinical, laboratory and radiological data did not correspond with the usual causes of eosinophilic myelitis. Abnormal mite antigen-specific IgE levels and features similar to Japanese cases of atopic myelitis suggested an allergic origin. Despite normal total IgE levels, this case may be the second case of atopic myelitis reported in a Caucasian patient. Striking differences with the first reported case are the absence of a typical history of atopy and normal total IgE levels. This case highlights that atopic myelitis should be considered in myelopathy occurring in Caucasian patients even in the absence of hyperIgEaemia.

Dissociation between numerosity and duration processing in aging and early Parkinson's disease

Numerosity and duration processing have been shown to be underlain by a single representational mechanism, namely an accumulator, and to rely on a common cerebral network located principally in areas around the right intraparietal sulcus. However, recent neuropsychological findings reveal a dissociation between numerosity and duration processing, which suggests the existence of partially distinct mechanisms. In this study, we tested the idea of partially common and distinct mechanisms by investigating, for the first time, both numerical and temporal processing abilities in non-demented Parkinsons disease (PD) patients known to suffer from duration impairment and in healthy elderly adults known to have impaired performance in duration tasks. The aim was to assess whether this impaired duration processing would extend to numerosity processing. The participants had to compare either the numerosity of flashed dot sequences or the duration of single dot displays. The results demonstrate an effect of aging on duration comparison, healthy elderly participants making significantly more errors than healthy young participants. Importantly, the performance of PD patients on the duration task was worse than that of the healthy young and elderly groups, whereas no difference was found for numerosity comparison. This dissociation supports the idea that partly independent systems underlie the processing of numerosity and duration.

Abdominal wall weakness and lumboabdominal pain revealing neuroborreliosis: a report of three cases.

Abstract: The authors report three cases of thoracic radiculoneuropathy disclosing neuroborreliosis. All three patients had low back and abdominal pain and two had marked abdominal wall paresis. EMG confirmed a motor involvement of the lower thoracic roots and CSF analysis revealed a lymphocytic meningitis in all three cases. Antibodies against Borrelia burgdorferi were present in both the serum and the CSF. A favourable outcome was obtained in all three patients with appropriate antibiotherapy. The differential diagnosis of this misleading presentation is discussed.

Symptoms of depression and anxiety after the disclosure of the diagnosis of Alzheimer disease.

Background and purpose: Many people fear that the disclosure of the diagnosis of Alzheimer disease (AD) to patients will prompt depressive symptoms or catastrophic reactions. We aimed to prospectively evaluate the modification of anxiety and depressive symptoms 3 months after the disclosure of the diagnosis of AD. Methods: A total of 100 consecutive newly diagnosed patients with AD (mild or moderate stage) and their caregivers were included. The evolution of symptoms of depression and anxiety was assessed with the Zung Self-Rating Depression Scale (Zung SDS) and the depression item of the Neuropsychiatric Inventory (NPI-d) and the anxiety item of the Neuropsychiatric Inventory (NPI-a). After 3 months, the caregivers were asked their opinions on the global effect of the disclosure using a Likert-type scale. Results: At 3 months, there was no significant change in the mean NPI-d (P = .87) and Zung SDS (P = .18) and a significant reduction in the NPI-a (P = .05). The NPI-d worsened in 22% of patients, improved in 22%, and remained unchanged in 56%. The NPI-a worsened in 12% of patients, improved in 33%, and remained unchanged in 54%. The caregivers rated the global effect of the disclosure as negative in 8%, neutral in 71%, and positive in 21% of patients. None of the patients or their proxies reported suicide attempts or catastrophic reactions. Conclusions: The disclosure of AD is safe in most cases and may improve anxiety. Symptoms of depression and anxiety worsen only in a minority of patients. The fear of depression or catastrophic reaction should not prevent clinicians to disclose the diagnosis of AD.

Validity of the Five-Word Test for the Evaluation of Verbal Episodic Memory and Dementia in a Memory Clinic Setting

Background: The five-word test (FWT) uses semantic clues to optimize the encoding and retrieval of 5 items. Our objective was to assess the validity of the FWT as a measure of episodic memory when compared with the Free and Cued Selective Reminding Test (FCSRT), and its ability to distinguish participants with any dementia and especially Alzheimer disease (AD) from those with only subjective complaints. Methods: Two hundred participants with Mini-Mental State Examination (MMSE) >15 were prospectively evaluated. The sum of the immediate and delayed free recalls of the FWT is called the free recall score (FRS). The sum of the immediate free, immediate cued, delayed free, and delayed cued recalls is called the total recall score (TRS). A total weighted score (TWS) is calculated using this formula: (free recalls × 2) + cued recalls. Results: The correlation between FRS and the free recall scores of the FCSRT and between TRS and the total recall scores of the FCSRT was significant (r s ranges from .74-.84, P < .001). Area under the receiver–operating characteristic (ROC) curves of the MMSE, FRS, TRS, and TWS were not statistically different. A TWS at a cutoff value ≤15 could discriminate any dementia from subjective complaints with a sensitivity of 75% and a specificity of 95.9% or AD from subjective complaints with a sensitivity of 90.2% and a specificity of 95.9%. Conclusion: The FWT is a valid test of verbal episodic memory. It is useful to discriminate dementia especially AD from isolated subjective complaints.

Clinical utility and applicability of biomarker-based diagnostic criteria for Alzheimer’s disease: a BeDeCo survey

We conducted a survey regarding the medical care of patients with dementia in expert settings in Belgium. Open, unrestricted and motivated answers were centralized, blindly interpreted and structured into categories. The report of the results was then submitted to the participants in subsequent plenary meetings and through email. Fourteen experts responded to the questionnaire, confirming that recent propositions to modify Alzheimer’s disease (AD) diagnostic criteria and options have stirred up debate among well-informed and dedicated experts in the field. The opinions were not unanimous and illustrate how difficult it is to find a standardized method of diagnosing this disease. The responses to the survey suggest that application of a step-by-step pragmatic method is used in practice. Only when the combination of clinical findings and classical structural neuro-imaging is insufficient for a diagnosis or suggests an atypical presentation, additional biomarkers are considered. Interestingly, few differences, if any, were observed between the use of biomarkers in MCI and in AD. In conclusion, the Belgian experts consulted in this survey were generally in agreement with the new diagnostic criteria for AD, although some concern was expressed about them being too “amyloidocentric”. Although the clinical examination, including a full neuropsychological evaluation, is still considered as the basis for diagnosis, most experts also stated that they use biomarkers to help with diagnosis.

Asymptomatic posterior reversible encephalopathy revealed by brain MRI in a case of axonal Guillain-Barré syndrome.

Posterior reversible encephalopathy syndrome (PRES) is a clinicoradiologic syndrome characterized by headaches, encephalopathy, visual disturbances and seizures, with a transient vasogenic oedema on brain imaging. The lesions are predominantly but not exclusively located in the posterior circulation system. The most frequent aetiologies include hypertension, cytotoxic or immunosuppressive drugs, sepsis, eclampsia or preeclampsia [1]. Some cases of PRES are described in association with Guillain-Barre syndrome (GBS) [2]. We report the case of a young woman who presented with a GBS, dysautonomia, and a brain MRI suggesting PRES but lacking any encephalic symptom suggestive of that syndrome. We discuss the relationship between, PRES, GBS and dysautonomia.

POLG2 deficiency causes adult-onset syndromic sensory neuropathy, ataxia and parkinsonism.

Mitochondrial dysfunction plays a key role in the pathophysiology of neurodegenerative disorders such as ataxia and Parkinsons disease. We describe an extended Belgian pedigree where seven individuals presented with adult‐onset cerebellar ataxia, axonal peripheral ataxic neuropathy, and tremor, in variable combination with parkinsonism, seizures, cognitive decline, and ophthalmoplegia. We sought to identify the underlying molecular etiology and characterize the mitochondrial pathophysiology of this neurological syndrome.

Parkinsonism and cognitive impairment due to a giant frontal arachnoid cyst and improving after shunting

Parkinsonism secondary to an arachnoid cyst appears to be very rare. We report the case of a 75-year-old woman who developed progressive gait and cognitive slowing during the preceding year. More recently, she complained of postural instability. Neurological examination revealed mild postural and action tremors of the upper limbs, along with moderate bilateral akinesia that was predominant on the left side, as well as slight rigidity of the left arm. Her face was hypomimic. Her gait was slow with short steps and no arm swing. Postural reflexes were severely reduced (Hoehn and Yahr score 3). The MDS-UPDRS Part 3 motor score was 47 (detailed in Online Resource). Her Mini Mental State Examination (MMSE) score was 25/30. She could name the months of the year backwards in 44 s. Phonemic and semantic verbal fluencies were markedly reduced (five words beginning with the letter ‘‘p’’ and seven animal names in 2 min). An extended neuropsychological evaluation showed a dysexecutive syndrome with impairment of mental flexibility and cognitive inhibition, abnormal slowing, weakness in the area of selective attention and deficit of free recall in episodic memory. Picture naming was normal. Treatment with a maximum dose of 600 mg levodopa over the course of 3 months did not improve motor symptoms. A brain MRI revealed a giant right frontal arachnoid cyst (size 95 9 65 9 58 mm) with a significant mass effect on the third and right lateral ventricles and the adjacent brain parenchyma, including the basal ganglia. Hyperintensity was seen in the left periventricular white matter on T2-weighted sequences (Fig. 1). A cyst-peritoneal shunt with an Orbis sigma valve (OSV2, Integra, France) was placed. Postoperatively, the patient showed dramatic improvement. Five weeks after shunting, gait was normal except for a mild reduction in arm swing. Postural reflexes were only mildly decreased (Hoehn and Yahr score 2). Mild akinesia and normal muscle tone were observed in the four limbs. The MDSUDRS Part 3 motor score was 19. The MMSE score was 28/30. She could name the months backwards in 22 s. Verbal fluency improved (11 words beginning with ‘‘p’’ and 15 animals in 2 min). Her husband confirmed marked improvement of cognitive functions in daily life. Brain computed tomography showed a significant decrease in the cyst volume (45 9 58 9 43 mm), with disappearance of the mass effect and of the abnormal signal of the white matter on the left side and a new 12 mm width left subdural fluid collection (Fig. 2), consistent with excessive drainage. Eight months after shunting, the neurological examination was normal. A brain CT showed the disappearance of the subdural fluid collection. The volume of the arachnoid cyst remained stable. Cognitive impairment of the frontal-subcortical type without aphasia and asymmetric parkinsonism that was predominant on the left were consistent with the right frontal location of the cyst. However, our patient displayed Electronic supplementary material The online version of this article (doi:10.1007/s13760-016-0632-3) contains supplementary material, which is available to authorized users.

Alzheimer's disease and driving: review of the literature and consensus guideline from Belgian dementia experts and the Belgian road safety institute endorsed by the Belgian Medical Association.

Alzheimer’s disease (AD) is a highly prevalent condition and its prevalence is expected to further increase due to the aging of the general population. It is obvious that the diagnosis of AD has implications for driving. Finally, driving discussions are also emotionally charged because driving is associated with independence and personal identity. However, it is not clear how to implement this in clinical practice and the Belgian law on driving is rather vague in its referral to neurodegenerative brain diseases in general nor does it provide clear-cut instructions for dementia or AD compared to for example driving for patients with epilepsy and as such does not prove to be very helpful. The present article reviews what is known from both literature and existing guidelines and proposes a consensus recommendation tailored to the Belgian situation agreed by both AD experts and the Belgian Road Safety Institute endorsed by the Belgian Medical Association. It is concluded that the decision about driving fitness should be considered as a dynamic process where the driving fitness is assessed and discussed early after diagnosis and closely monitored by the treating physician. The diagnosis of AD on itself definitely does not imply the immediate and full revocation of a driving license nor does it implicate a necessary referral for a formal on-road driving assessment. There is no evidence to recommend a reduced exposure or a mandatory co-pilot. A MMSE-based framework to trichotomise AD patients as safe, indeterminate or unsafe is presented. The final decision on driving fitness can only be made after careful history taking and clinical examination, neuropsychological, functional and behavioral evaluation and, only for selected cases, a formal assessment of driving performance.