Episodic epileptic verbal auditory agnosia in Landau Kleffner syndrome treated with combination diazepam and corticosteroids.

We report 2 pediatric patients who presented initially with seizures followed by subacute language regression characterized by a verbal auditory agnosia. These previously normal children had no evidence of expressive aphasia during their symptomatic periods. Further, in both cases, auditory agnosia was associated with sleep-activated electroencephalographic (EEG) epileptiform activity, consistent with Landau-Kleffner syndrome. However, both cases are unique since the episodic auditory agnosia and sleep-activated EEG epileptiform activity rapidly responded to combination therapy with pulse benzodiazepine and corticosteroids. Further, in each case, recurrences were characterized by similar symptoms, EEG findings, and beneficial responses to the pulse benzodiazepine and corticosteroid therapy. These observations suggest that pulse combination high-dose corticosteroid and benzodiazepine therapy may be especially effective in Landau-Kleffner syndrome.

The effect of nicotine on visuospatial attention in chronic spatial neglect depends upon lesion location.

The deficit to reorient attention from ipsilesional to contralesional space is one key feature of the spatial neglect syndrome. As previous studies suggest that reorienting of visuospatial attention is modulated by cholinergic neurotransmission, we investigated whether cholinergic stimulation with nicotine (Nicorette 2 mg, Pharmacia/Pfizer, Helsingborg, Sweden) facilitates attentional reorienting in spatial neglect patients. Nine nonsmoking patients with stable neglect symptoms were investigated in a within-subject cross-over design. We used a location-cueing paradigm and analysed reaction time (RT) differences between validly and invalidly cued, as well as between neutrally cued and uncued targets as a function of hemifield and drug. Moreover, since the nicotine effect is mediated by parietal brain areas in healthy subjects, we tested whether lesion location influences the pharmacological effect. Nicotine speeded RTs in valid and invalid trials nonspecifically, without modulating the validity effect in the location-cueing task in the whole group of patients. Lesion-symptom mapping revealed a relationship between lesion site and the pharmacological effect on reorienting to contralesional space in right parietal and temporal brain regions. We conclude that in patients with chronic spatial neglect the performance in the location-cueing paradigm can be modulated by a cholinergic stimulant provided that the lesion spares right parietal and temporal cortex.

Anosognosia for hemiplegia after stroke is a multifaceted phenomenon: a systematic review of the literature.

Anosognosia is the lack of awareness or the underestimation of a specific deficit in sensory, perceptual, motor, affective or cognitive functioning due to a brain lesion. This self-awareness deficit has been studied mainly in stroke hemiplegic patients, who may report no deficit, overestimate their abilities or deny that they are unable to move a paretic limb. In this review, a detailed search of the literature was conducted to illustrate clinical manifestations, pathogenetic models, diagnostic procedures and unresolved issues in anosognosia for motor impairment after stroke. English and French language papers spanning the period January 1990-January 2007 were selected using PubMed Services and utilizing research words stroke, anosognosia, awareness, denial, unawareness, hemiplegia. Papers reporting sign-based definitions, neurological and neuropsychological data and the results of clinical trials or historical trends in diagnosis were chosen. As a result, a very complex and multifaceted phenomenon emerges, whose variable behavioural manifestations often produce uncertainties in conceptual definitions and diagnostic procedures. Although a number of questionnaires and diagnostic methods have been developed to assess anosognosia following stroke in the last 30 years, they are often limited by insufficient discriminative power or a narrow focus on specific deficits. As a consequence, epidemiological estimates are variable and incidence rates have ranged from 7 to 77% in stroke. In addition, the pathogenesis of anosognosia is widely debated. The most recent neuropsychological models have suggested a defect in the feedforward system, while neuro-anatomical studies have consistently reported on the involvement of the right cerebral hemisphere, particularly the prefrontal and parieto-temporal cortex, as well as insula and thalamus. We highlight the need for a multidimensional assessment procedure and suggest some potentially productive directions for future research about unawareness of illness.

Treatment of Susac syndrome with gamma globulin and corticosteroids.

Susac syndrome is a rare vasculopathy characterized by visual, hearing, and cognitive dysfunction. Optimal treatment is unknown, but many patients require chemotherapy to control disease activity. We describe two patients with Susac syndrome and their response to intravenous immune globulin (IVIg) and corticosteroids. Both patients improved following acute treatment with IVIg and intravenous methylprednisolone (IVMP), and no further relapses were observed. One patient showed significant improvement in hearing and MRI lesions shortly following acute treatment. Treatment with IVIg and corticosteroids provides a therapeutic option that avoids the toxicities of chemotherapy and suggests the possible importance of pathologic antibodies in the pathogenesis of Susac syndrome.

[Clinical characteristics and long-term prognosis of Landau-Kleffner syndrome].

OBJECTIVE: To investigate the clinical and electroencephalographic (EEG) characteristics, therapeutic response and long-term prognosis of Landau Kleffner syndrome (LKS). METHODS: The clinical and EEG data of 10 children with LKS were analyzed, and therapeutic response and long-term outcome were followed up. RESULTS: The age of onset was from 2 to 10.5 years of age. All patients had acquired aphasia, characterized by verbal auditory agnosia. All patients had epileptic seizures. Partial motor seizures during sleep occurred in 8 patients, and other seizure type including atypical absence seizure and generalized tonic-clonic seizure were also observed. Psychological and behavioral abnormalities occurred in 9 patients. There were no abnormalities of hearing and neuro-imaging tests in all patients, and family histories were negative. All the patients had EEG abnormalities. Focal spike and waves of temporal lobe were recorded in 9 patients. Electrical status epilepticus during sleep (ESES) was observed on Video-EEG (VEEG) monitoring in 4 patients. Anti-epileptic drugs (AEDs) showed favorable effects on epileptic seizures, but no effects on aphasia. All patients responded to corticosteroid, and got language improved. Eight patients were followed up for long-term outcome. All patients were seizure free, while the level of language development was abnormal in 5 patients. The VEEG follow-up was conducted in 6 patients. Continuous epileptic discharges in slow sleep recurred in 2 patients after the discontinuation of steroid therapy. CONCLUSIONS: LKS is one of the childhood epileptic encephalopathy, and acquired aphasia and epileptic seizures are two main clinical characteristics. Aphasia is characterized by verbal auditory agnosia. Psychological and behavioral abnormalities are very common in children with LKS. Focal epileptic discharges were often located in temporal area, and usually generalized, and could be continuous during sleep. AEDs could control seizure but had no effects on aphasia. Early use of full dose corticosteroids could improve the language significantly. Long-term follow up showed that language impairments often remained, but the outcome in terms of EEG and epileptic seizure was good.

Response of emotional unawareness after stroke to antidepressant treatment.

OBJECTIVE: Unawareness of impairment (anosognosia) is a phenomenon associated with right hemisphere lesions. Unawareness of emotion has rarely been studied. METHODS: Patients (N = 50) with poststroke major depression were administered the Toronto, Ontario, Canada, Alexithymia Scale to assess impairment in identifying feelings (F1), describing feelings (F2), and externally oriented thinking (F3). After eight weeks of treatment with sertraline or fluoxetine, patients were reassessed. RESULTS: Alexithymia was significantly associated with right hemisphere lesions. Patients with alexithymia had a significant improvement in identifying and describing feelings, but not in externally oriented thinking. In addition, cognitive functions improved after antidepressant treatment in patients without alexithymia with left lesions only. On the contrary, functional activities of daily living and depressive symptoms improved both in patients with alexithymia and those without alexithymia. CONCLUSIONS: The unawareness of emotions is a common impairment after right hemisphere stroke. This disorder may be significantly improved by antidepressant treatment.

Primary central nervous system angiitis, amyloid angiopathy, and Alzheimer's pathology presenting with Balint's syndrome.

We report a patient who presented with Balint's syndrome as a manifestation of primary central nervous system angiitis. Clinical findings included simultanagnosia, optic ataxia, and optic apraxia. Pathologic evaluation demonstrated amyloid angiopathy and Alzheimer's plaques. The presence of primary central nervous system angiitis along with amyloid angiopathy and Alzheimer's plaques may not be coincidental. Angiitis may be a foreign body reaction to A4 amyloid deposition.

Diazepam therapy of verbal auditory agnosia.

A 7-year-old girl suffered from a gradual loss of her ability to understand speech. But she had normal hearing and understood various environmental sounds. Brain computerized axial tomography showed normal, but electroencephalogram revealed spike and wave activity from temporal leads, especially on the left side. She was diagnosed as having verbal auditory agnosia. The treatment consisted of diazepam therapy. One year after the onset, her impaired auditory comprehension dramatically improved with the use of diazepam. The dichotic listening test revealed a left ear advantage for both environmental sounds and spoken words. The results seemed to suggest that in this patient the right hemisphere might be functioning as a speech center instead of the left one.

Regional metabolism in microsmic patients with schizophrenia.

The cerebral glucose metabolism of eight patients with schizophrenia and an olfactory agnosia was compared with that of eight normosmic patients with schizophrenia and eight normal controls. Since all patients were scanned while on their current medication regimen, the duration and dosage of the medication of the two patient groups were compared. Similarly, duration and dosage were correlated with absolute regional metabolic rates. No significant effects were found in these analyses. The patients with schizophrenia had significantly lower rates of frontal metabolism than the normal controls. However, the patients with schizophrenia and an olfactory agnosia had a lower right basal ganglia and thalamic metabolism than the normosmic patients with schizophrenia.