Carla Cristina Adda

Prospective memory and mesial temporal epilepsy associated with hippocampal sclerosis

Episodic memory impairment is commonly observed in patients with epilepsy associated with mesial temporal sclerosis (MTS). Prospective memory (PM) is a set cognitive abilities that allow future performance of a present intention, in response to time- or event-based evocation cues, that trigger the intended action at the appropriate time. PM has not been evaluated in mesial temporal sclerosis. We evaluated the role of right and left hippocampal lesions on performance in both the retrospective and prospective PM components in patients with epilepsy secondary to mesial temporal sclerosis and correlated with performance in traditional neuropsychological tests, as well as with self-perception of memory impairment. We tested the hypotheses that a hippocampal lesion impacts on the prospective components of PM, and that a left-sided lesion had a greater impact on performance in the prospective component of PM than a right-sided lesion. We evaluated PM in 26 patients with right MTS, 22 left MTS patients, and 26 age-gender and education matched controls. The prospective component of PM was impaired in both patient groups, with both a lesion (patients performed significantly worse in the PM battery) and laterality effect (left MTS patients performed significantly worse than right MTS patients in the PM battery). Performance in the prospective component of the PM battery correlated with long-term delay performance in episodic verbal memory and self-perception of memory impairment in the left MTS group. The retrospective component was impaired in left MTS patients. Impaired performance was not accounted for solely by depression, anxiety or an antiepileptic drug effect. We conclude that mesial temporal lobe structures, including the hippocampus, play an important role in both the prospective and retrospective components of PM processes in tasks involving long delay intervals.

Epilepsy syndromes associated with hypothalamic hamartomas

PURPOSE Hypothalamic hamartoma (HH) related epilepsy presents with gelastic seizures (GS), other seizure types and cognitive deterioration. Although seizure origin in GS has been well established, non-GS are poorly characterized. Their relationship with the HH and cognitive deterioration remains poorly understood. We analyzed seizure type, spread pattern in non-GS and their relationship with the epileptic syndrome in HH. METHODS We documented all current seizure types in six adult patients with HH-epilepsy with video-EEG monitoring, characterized clinical-electrographic features of gelastic and non-gelastic seizures and correlated these findings with cognitive profile, as well as MRI and ictal SPECT data. RESULTS Only four seizure types were seen: GS, complex partial (CPS), tonic seizures (TS) and secondarily generalized tonic-clonic seizures (sGTC). An individual patient presented either CPS or TS, but not both. GS progressed to CPS or TS, but not both. Ictal patterns in GS/TS and in GS/CPS overlapped, suggesting ictal spread from the HH to other cortical regions. Ictal SPECT patterns also showed GS/TS overlap. Patients with GS-CPS presented a more benign profile with preserved cognition and clinical-EEG features of temporal lobe epilepsy. Patients with GS-TS had clinical-EEG features of symptomatic generalized epilepsy, including mental deterioration. CONCLUSIONS Video-EEG and ictal SPECT findings suggest that all seizures in HH-related epilepsy originate in the HH, with two clinical epilepsy syndromes: one resembling temporal lobe epilepsy and a more catastrophic syndrome, with features of a symptomatic generalized epilepsy. The epilepsy syndrome may be determined by HH size or by seizure spread pattern.

Low prevalence but high specificity of material-specific memory impairment in epilepsy associated with hippocampal sclerosis

Material‐specific memory impairment is used as a lateralizing tool in the evaluation of temporal lobe epilepsy. Lateralizing ability of material‐specific memory deficits in temporal lobe epilepsy remains controversial.

Narcolepsy and depression

Narcolepsy main symptoms include excessive daytime sleepiness and cataplexy. Its chronic course is accompanied by psychosocial impairment added to the difficulties and side effects of stimulants and tricyclics long term use. Depressive complaints are occasionally reported. The aim of this paper was to evaluate objectively the possibility of depression in a sample of 12 narcoleptics (7F; 5M), with mean age of 53 years (12 years SD), using the Beck Depression Inventory (BDI) and the Hamilton Rating Scale for Depression (HAM-D). The results showed absence of depressive disorder in 75.0% of the cases according to BDI (or 58.3% according to HAM-D). The remaining patients had mild depression (only one patient presented major depression). The findings showed no correlation between narcolepsy and major depression.

The benign spectrum of hypothalamic hamartomas: Infrequent epilepsy and normal cognition in patients presenting with central precocious puberty

PURPOSE Hypothalamic hamartoma (HH) is the main structural cause of central precocious puberty (CPP). HH is frequently associated with cognitive impairment and epileptic encephalopathies. Disease severity in case series from neurology services may be biased towards more neurologically impaired patients. AIM To perform a prospective cognitive evaluation in patients with HH presenting with CPP in an endocrinology outpatient clinic setting. METHODS We evaluated fifteen consecutive patients with CPP due to HH presenting to an endocrinology outpatient clinic. CPP was diagnosed at a median age of 0.7 yr (0.4-7 yr). Mean age at neurologic evaluation was 13.9 yrs. Eight patients (53.3%) were male. Epileptic seizures occurred in 5/15 (33%) patients. Two patients presented a single unprovoked seizure (SUS). Three patients were diagnosed with epilepsy. Cognitive evaluation, using age-appropriate Wechsler Intelligence Scale, was performed in 11 patients. RESULTS All patients without epilepsy, including two patients with a history of a SUS, had normal neurologic and cognitive evaluation. Epilepsy and SUS were only seen in patients with sessile HH. Three patients with epilepsy presented cognitive or behavioral findings. Reduced intelligence quotients (IQ), in the borderline range, were noted in both patients with epilepsy who underwent full cognitive evaluation. We found no significant correlation between HH diameter or shape and mean full-scale IQ. CONCLUSIONS Patients who presented with isolated CPP without epilepsy displayed normal cognition when evaluated after a mean period of 13 years. Occurrence of epilepsy, seen in a minority of patients, but not of a single seizure, was associated with mild cognitive deficit and behavioral disturbances in this case series.

Association among depression, cognitive impairment and executive dysfunction after stroke

The relationship between depression and cognitive impairment, frequent after stroke, is complex and has not been sufficiently elucidated. Objective To review the relationship between post-stroke depression and cognitive impairment. Methods We performed a PubMed database search spanning the last ten years, using the terms post-stroke depression, cognitive dysfunction, cognitive impairment and neuropsychological tests. Our target studies were original quantitative studies that investigated the relationship between post-stroke depression (PSD) and cognitive impairment in stroke patients. Articles published in English, Spanish, Italian and Portuguese were considered. Selection criteria were the use of neuropsychological tests to assess cognitive function, and of either instruments to diagnose major depression, or scales to assess depressive symptoms, within the first three months after stroke. Results Six original quantitative studies fulfilled the criteria. The prevalence of PSD within the first three months after stroke ranged from 22% to 31%. Incidence ranged from 25% to 27% and was evaluated in only two studies. PSD was associated with increased cognitive impairment. Cognitive impairment was reported in 35.2% to 87% of the patients. Post-stroke cognitive deficits were reported mostly in executive function, memory, language, and speed of processing. Conclusion Executive dysfunction and depression occur in stroke survivors, are frequently coexistent, and also associated with worse stroke prognosis. Healthcare professionals need to address and provide adequate treatment for depression and executive dysfunctions in stroke patients early in the first three months after stroke. Future studies should evaluate the efficacy of programs evaluating the early detection and treatment of PSD and executive dysfunction in stroke survivors.

Language impairment in Huntington's disease

Language alterations in Huntingtons disease (HD) are reported, but their nature and correlation with other cognitive impairments are still under investigation. This study aimed to characterize the language disturbances in HD and to correlate them to motor and cognitive aspects of the disease. We studied 23 HD patients and 23 controls, matched for age and schooling, using the Boston Diagnostic Aphasia Examination, Boston Naming Test, the Token Test, Animal fluency, Action fluency, FAS-COWA, the Symbol Digit Modalities Test, the Stroop Test and the Hooper Visual Organization Test (HVOT). HD patients performed poorer in verbal fluency (p<0.0001), oral comprehension (p<0.0001), repetition (p<0.0001), oral agility (p<0.0001), reading comprehension (p=0.034) and narrative writing (p<0.0001). There was a moderate correlation between the Expressive Component and Language Competency Indexes and the HVOT (r=0.519, p=0.011 and r=0.450, p=0.031, respectively). Language alterations in HD seem to reflect a derangement in both frontostriatal and frontotemporal regions.

Psychological assessment in patients with phobic postural vertigo

UNLABELLED Phobic postural vertigo (PPV) is a frequent diagnosis which can be challenging to treat. OBJECTIVE To investigate the presence of psychiatric disturbances in patients with PPV; to assess the psychological status of patients using adaptive diagnosis; to verify possible correlations between severity of psychiatric disturbance and adaptive efficacy. METHOD A total of nineteen subjects were assessed and two instruments applied: the Primary Care Evaluation of Mental Disorders Questionnaire (PRIME-MD) and the Adaptive Operationalized Diagnostic Scale (AODS), and results from both tests were compared. RESULTS Fourteen patients presented with mood disorder and thirteen with anxiety. All patients presented compromised adaptive efficacy. Correlation was found between overall outcome on the PRIME and the AODS (tau= -0.42, p=0.027), Separate analysis revealed correlation between results of the AODS and anxiety disorders (tau= -0.45, p=0.018) but not with mood disorders (tau= -0.36, p=0.054). CONCLUSION Adaptive compromise was observed in individuals with PPV which was shown to be associated to psychiatric disorders.

Torre de Londres e Torre de Hanói: contribuições distintas para avaliação do funcionamento executivo

OBJECTIVE: To produce theoretical study about comparisons of cognitive and methodological aspects involved in the performance of Towers of Hanoi and London in normal individuals. METHOD: Narrative review of the literature. RESULTS: The performances in these instruments do not share the expected variance due to the apparent similarities in your structure and solution demand. Correlations significant, however moderate, among the two towers performance, were found reflecting the average of 75% of the nonshared variance. CONCLUSIONS: Both towers recruit different executive processes for task solution and cannot be used as exchangeable instruments.

Language and visuospatial impairment in a case of crossed aphasia

Crossed aphasia in dextrals (CAD) constitutes an interesting model for understanding the lateralisation and interaction of language with other cognitive functions in the brain. The authors present a study of a right-handed patient with Wernickes aphasia following a right hemisphere cerebrovascular accident, who also had impairment in visuospatial skills. Although the patient presented a remarkable improvement in language symptoms on longitudinal follow-up, the combination of linguistic, visuospatial, and attentional impairments ultimately resulted in a persisting inability to perform complex tasks. The study of mild residual disturbances can improve our understanding of the interaction of language with other cognitive functions, going some way towards explaining the particular features found in CAD.