Neurocognitive impairment in Whipple disease with central nervous system involvement.

Young-onset dementias pose a major challenge to both clinicians and researchers. Cognitive decline may be accompanied by systemic features, leading to a diagnosis of "dementia plus" syndromes. Whipple disease is a rare systemic illness characterized by arthralgias, chronic diarrhea, weight loss, fever, and abdominal pain. Central nervous system involvement, including severe cognitive deterioration, may precede systemic manifestations, appear during the course of the disease, or even be the only symptom. We report a previously highly functional 48-year-old man whom we first suspected of having early-onset neurodegenerative dementia but then diagnosed with Whipple disease based on a detailed clinical and laboratory evaluation. Initial neuropsychological evaluation revealed marked impairment in the patient's fluid intelligence and severe cognitive deficits in his information processing speed, complex attention, memory, visuomotor and construction dexterities, problem solving, and executive functions. At neuropsychological follow-up 21 months later, his information processing speed had improved only slightly and deficits persisted in his other cognitive functions. Repeat brain magnetic resonance imaging at that time showed that he had responded to antibiotic treatment. Because Whipple disease can cause young-onset "dementia plus" syndromes that may leave patients with neurocognitive deficits even after apparently successful treatment, we recommend comprehensive neuropsychological assessment for early detection of residual and reversible cognitive processes and evaluation of treatment response.

Trail Making Test error analysis in classic motor neuron disease.

The use of non-traditional scores in neuropsychological assessment allows for pattern analysis of test performance, commonly referred to as Quantified Process Approach (QPA). In the present study, the QPA was taken to study error rates on the Trail Making Test (TMT) in 26 non-demented patients with classic motor neuron disease (cMND), who commonly present with impaired cognitive flexibility, and 26 matched healthy controls. Between-group comparisons revealed that cMND patients exhibited higher total error rates on the TMT Part B (TMT-B) relative to controls (p < 0.001), though no significant associations were noted between TMT errors and measures of attention and executive function obtained using the Stroop Neuropsychological Screening Test, the Wechsler Adult Intelligence Scale Digit Symbol and Digit Span subtests and the Wisconsin Card Sorting Test. Moreover, the percentage of cMND patients with normal TMT-B time-to-completion who committed at least one error (either sequential or perseverative errors) in TMT-B was significantly higher compared to controls (p = 0.005). These findings suggest that error analysis using the QPA may increase the clinical utility of TMT and should be considered in addition to time-to-completion scores, in the neuropsychological assessment of patients with cMND.

Cognitive deficits presenting as psychiatric symptoms in a patient with Moyamoya disease.

The case of a middle-aged man who had intraventricular hemorrhages secondary to Moyamoya disease (MMD), and initially, presented with psychiatric symptoms which did not respond to treatment is described. Neuropsychological assessment showed underlying significant cognitive deficits, mostly of complex attention and speed of information processing, visuospatial and constructional abilities, verbal and nonverbal memory, and executive functions. These deficits remained stable or slightly improved in follow-up assessments. Cognitive dysfunction should be suspected in case of psychiatric or psychiatric-related symptoms in MMD patients. This case study in Greece highlights the range of preserved and impaired cognitive functions in adult MMD by means of repeated neuropsychological evaluations in which a broad range of cognitive abilities were assessed.

Unusual cases of multiple symmetrical lipomatosis with neurological disorders.

Multiple symmetrical lipomatosis (MSL) is a rare disorder of unknown etiology defined as the presence of multiple and symmetrical fatty accumulations, usually involving the upper trunk, neck and head. Frequently associated findings include diabetes mellitus, hyperlipidemia, liver disease, hypothyroidism and polyneuropathy of unknown origin, but nevertheless, there are published reports of cognitive disorders in patients with MSL. We describe two unusual cases (38-year-old and 45-year-old Greek men) of MSL who presented with polyneuropathy and memory disorders. This is the first description of memory disorders in patients with MSL. We propose that Mini-Mental State Examination and assessment of cognitive functions should be performed for all patients with MSL. The underlying mechanism in our patients remains unknown, and this question should be the subject of a future study.

Folate and vitamin B12 levels in levodopa-treated Parkinson's disease patients: their relationship to clinical manifestations, mood and cognition.

We tested the hypothesis that mood, clinical manifestations and cognitive impairment of levodopa-treated Parkinson's disease (PD) patients are associated with vitamin B12 and folate deficiency. To this end, we performed this cross-sectional study by measuring serum folate and vitamin B12 blood levels in 111 consecutive PD patients. Levodopa-treated PD patients showed significantly lower serum levels of folate and vitamin B12 than neurological controls, while depressed patients had significantly lower serum folate levels as compared to non-depressed. Cognitively impaired PD patients exhibited significantly lower serum vitamin B12 levels as compared to cognitively non-impaired. In conclusion, lower folate levels were associated with depression, while lower vitamin B12 levels were associated with cognitive impairment. The effects of vitamin supplementation merit further attention and investigation.

Depression and anxiety in epilepsy: the association with demographic and seizure-related variables.

BACKGROUND: Depression and anxiety are common psychiatric symptoms in patients with epilepsy, exerting a profound negative effect on health-related quality of life. Several issues, however, pertaining to their association with psychosocial, seizure-related and medication factors, remain controversial. Accordingly, the present study was designed to investigate the association of interictal mood disorders with various demographic and seizure-related variables in patients with newly-diagnosed and chronic epilepsy. METHODS: We investigated 201 patients with epilepsy (51.2% males, mean age 33.2 +/- 10.0 years, range 16-60) with a mean disease duration of 13.9 +/- 9.5 years. Depression and anxiety were assessed in the interictal state with the Beck Depression Inventory, 21-item version (BDI-21) and the state and trait subscales of the State-Trait Anxiety Inventory (STAI-S and STAI-T), respectively. The association of mood disorders with various variables was investigated with simple and multiple linear regression analyses. RESULTS: High seizure frequency and symptomatic focal epilepsy (SFE) were independent determinants of depression, together accounting for 12.4% of the variation of the BDI-21. The STAI-S index was significantly associated with the type of epilepsy syndrome (SFE). Finally, high seizure frequency, SFE and female gender were independent determinants of trait anxiety accounting for 14.7% of the variation of the STAI-T. CONCLUSION: Our results confirm the prevailing view that depression and anxiety are common psychological disorders in epileptics. It is additionally concluded that female gender, high seizure frequency and a symptomatic epilepsy syndrome are independent risk factors for the development of anxiety and/or depression.

Add-on lamotrigine treatment and plasma glutamate levels in epilepsy: relation to treatment response.

We investigated the effects of add-on lamotrigine treatment on plasma glutamate (Glu) levels, in 29 epileptic patients. Plasma Glu levels were determined by high-performance liquid chromatography at baseline and at 1 and 3 months post-treatment. In patients with a seizure reduction of > or = 66% a decrease of Glu at month 1 was noted, followed by return to baseline levels at month 3. In the remaining patients a gradual increase of Glu was noted throughout the 3 months of the study. The above findings indicate that an excellent clinical response to add-on lamotrigine may be characterized by a statistically significant, yet transient decrease of plasma Glu levels, while increasing Glu levels may accompany a response that is moderate at best. The combination of lamotrigine with valproate was more frequent in patients with excellent clinical response and tended to result in glutamate decrease.

Patterns of depressive symptoms in epilepsy.

OBJECTIVE: The purpose of this study was to determine the nature and extent of depressive symptoms among patients with epilepsy. METHODS: Ninety patients were investigated over a three-month period: 42 were suffering from generalized epilepsy, 29 from focal epilepsy and 19 from undetermined epilepsy. All completed the Zung self-rating scale for assessment of the depressive symptoms. RESULTS: Sixty-seven patients felt stigmatized because of epilepsy (67%): 73.6% in the undetermined epilepsy group, 55.1% in the focal epilepsy group and 88% in the generalized epilepsy group. Moreover, among the 90 epileptic patients studied, symptoms of irritability, indecisiveness, personal devaluation and emptiness showed a constant increasing trend for their presence from the undetermined epilepsy group through the generalized epilepsy group to the focal epilepsy group. CONCLUSIONS: These findings indicate that although the focal epilepsy patients felt less stigmatized, they did not differ greatly in terms of depressive symptoms, in relation to the undetermined epilepsy and generalized epilepsy patients.

The stroop effect in Greek healthy population: normative data for the Stroop Neuropsychological Screening Test.

The Stroop Test is a quick and frequently used measure in screening for brain damage, dysfunction of selective attention, and cognitive flexibility. The purpose of the present study is to provide normative data for Trenerry's Stroop Neuropsychological Screening Test (SNST) in a sample of 605 healthy Greek participants (age range: 18-84 years, education range: 6-18 years). Results revealed that age and education significantly contributed to SNST scores, accounting for a significant proportion of variance in time needed to complete the color task and in the interference Color-Word score. Performance on most of the measures decreases with increasing age and lower levels of education. Normative data stratified by age and education for the Greek adult population are provided as a useful set of norms for clinical practice.

Patterns of depressive symptoms in epilepsy / Perfis de sintomas depressivos na epilepsia

Objective: The purpose of this study was to determine the nature and extent of depressive symptoms among patients with epilepsy. Methods: Ninety patients were investigated over a three-month period: 42 were suffering from generalized epilepsy, 29 from focal epilepsy and 19 from undetermined epilepsy. All completed the Zung self-rating scale for assessment of the depressive symptoms. Results: Sixty-seven patients felt stigmatized because of epilepsy (67%): 73.6% in the undetermined epilepsy group, 55.1% in the focal epilepsy group and 88% in the generalized epilepsy group. Moreover, among the 90 epileptic patients studied, symptoms of irritability, indecisiveness, personal devaluation and emptiness showed a constant increasing trend for their presence from the undetermined epilepsy group through the generalized epilepsy group to the focal epilepsy group. Conclusions: These findings indicate that although the focal epilepsy patients felt less stigmatized, they did not differ greatly in terms of depressive symptoms, in relation to the undetermined epilepsy and generalized epilepsy patients. .

Objetivo: O objetivo deste estudo foi determinar a natureza e extensão dos sintomas depressivos entre pacientes com epilepsia. Métodos: Noventa pacientes foram avaliados durante o período de três meses: 42 apresentando epilepsia generalizada, 29 com epilepsia focal e 19 com epilepsia indeterminada. Todos completaram a auto-escala de Zung para avaliação de sintomas depressivos. Resultados: Sessenta e sete pacientes se sentiam estigmatizados por causa da epilepsia (67%), sendo 73,6% do grupo epilepsia indeterminada, 55,1% do grupo epilepsia focal e 88% do grupo epilepsia generalizada. Além disto, entre os 90 pacientes epilépticos estudados, os sintomas de irritabilidade, dificuldade para tomar decisões, baixa estima pessoal e sensação de vazio mostraram-se presentes de forma crescente entre os grupos de epilepsia indeterminada, epilepsia generalizada e epilepsia focal. Conclusões: Estes achados indicam que embora os pacientes com epilepsia focal se sintam menos estigmatizados, eles não diferiram de maneira considerável daqueles com epilepsia indeterminada e epilepsia generalizada em termos de sintomas depressivos. .