Progressive topographical disorientation: a case of focal Alzheimer's disease.

We describe a follow-up study of a patient with a selective, progressive impairment of topographical orientation. The patient's topographical difficulties were evident only in unfamiliar surroundings at the beginning of the observation period but later on they were observed even at home. Serial neuropsychological tests demonstrated a progressive impairment of visuospatial abilities with sparing of the other cognitive domains; only at the last assessment, about six years after early disturbances and three years after the first evaluation, the patient developed the typical cognitive impairments of Alzheimer's disease (AD). This case represents a focal variant of AD not previously described and suggests that the neuronal pathways underlying spatial orientation may be selectively damaged by the degenerative process.

Effects of switching from an AChE inhibitor to a dual AChE-BuChE inhibitor in patients with Alzheimer's disease.

OBJECTIVE: Cholinesterase (ChE) inhibitors are the only medications approved for the treatment of Alzheimer's disease (AD). The features of ChE inhibitors differ considerably. In addition to acetylcholinesterase (AChE) inhibition, rivastigmine also inhibits butrylcholinesterase (BuChE), providing dual AChE and BuChE inhibition. An observational study was performed to determine the response in routine clinical practice to switching AD patients to rivastigmine from a selective AChE inhibitor when that treatment no longer delivered a satisfactory clinical response. RESEARCH DESIGN AND METHODS: A prospective, multicentre, 3-month observational trial in patients with mild to moderately severe AD (adjusted Mini Mental State Examination [MMSE] score 10-26) deteriorating (at least 2 adjusted MMSE points in last 6 months) on selective AChE inhibitor treatment. Adjusted MMSE, activities of daily living (ADL) and instrumental activities of daily living (IADL), the Zarit caregiver burden and global function (short Clinical Global Impression of Change, CGIC) scores were noted before the switch and 3 months after the switch. RESULTS: 225 patients entered the study. The switches made were from donepezil to rivastigmine in (D-R) in 188 patients, galantamine to rivastigmine (G-R) in 33 patients and donepezil to galantamine (D-G) in four patients. Ten patients discontinued due to adverse events and eight for other reasons. More than half of the switches were within 36 hours of a patient's first treatment visit. In the D-R and G-R groups, 67.7% and 66.7% of patients responded (CGIC score < or = 4), respectively. In non-responders, worsening (CGIC score 5-7) was mild in approximately 80% or more of patients. Adjusted MMSE improved after the switch from both donepezil and galantamine to rivastigmine (+0.69 +/- 3.2, p = 0.008 and +0.6 +/- 1.6, p = 0.05, respectively). Mean ADL, IADL, and Zarit scores remained stable. The proportion of patients on concomitant antipsychotic therapy diminished by 30.5% and benzodiazepines were discontinued in all patients, except one. CONCLUSIONS: AD patients deteriorating on selective AChE inhibitor treatment can benefit from switching to a dual AChE-BuChE inhibitor, such as rivastigmine, in terms of stabilization of disease, improvement in cognitive function and reduction in the burden of concomitant psychoactive treatment. The switch was well tolerated. Confirmation of these results is required in a controlled study.

Immunoglobulins i.v.: a new approach to the treatment of Guillain-Barré syndrome.

We report the results obtained with intravenous immunoglobulin (IVGG) in 15 patients suffering from Guillain Barré syndrome (GBS) with substantial motor damage. All of them met the NINCDS criteria for a diagnosis of certain GBS and were graded using a specific scale on entry, after 2 weeks, one month, two months, six months and one year. The clinical data were correlated to neurophysiological results. The patients were treated within the seventh day of the disease with IVGG (0.4 g/kg/day) for 5 days. Complete recovery was obtained in all but 2 patients died. We measured the mean time taken to improve one grade of the evaluation scale and the mean time taken to achieve walking unassisted. We obtained 11 days and 14 days respectively, that is, times significantly shorter than those reported in studies employing plasma exchange. No adverse effect was found except in one case. No relapse was observed. Even the patients with more clinical and neurophysiological damage had a rapid recovery. We advocate IVGG therapy for GBS, as it is effective, safe and easy to use.

Simultaneous occurrence of tetrasomy 21 and trisomy 8 in a patient with early blastic metamorphosis of chronic myeloproliferative disorder.

We report an unusual hyperdiploid karyotype characterized by the simultaneous occurrence of tetrasomy 21 and trisomy 8 detected during early blastic evolution of a BCR-ABL-negative chronic myeloproliferative disorder. Blast cells from this patient showed a striking response to all-trans-retinoic acid (ATRA)-induced differentiation as evaluated by CD15 expression following in vitro exposure to this inducer. Our report represents the first description of such a composite karyotype in human hematologic malignancies.

Early blastic transformation of a myeloproliferative disorder with t(8;21) and progressive aberrations of chromosome 8.

We describe a case of a patient affected by a chronic myeloproliferative disorder with t(8;21)(q22;q22) and trisomy 8 at diagnosis. At the time of blastic metamorphosis, 2 months later, trisomy 8 metaphases were significantly reduced, while a predominance of t(8;21) was present. Finally, in the phase of leukemic regrowth following chemotherapy administration, monosomy 8 associated with der(21)t(8;21) was the predominant cytogenetic abnormality.

Trisomy 4 and ring chromosome in a patient with acute myelomonocytic leukemia.

We describe a patient with acute myelomonocytic leukemia (AMML) in whom cytogenetic analysis revealed trisomy 4 associated with a ring chromosome. In addition, in a cytogenetically unrelated clone, trisomy 8 and 5q- abnormalities were detectable. The possibility of a subclinical myelodysplastic syndrome preceding the onset of AML is discussed on the basis of the morphological and cytogenetic findings.

Plasma exchange and i.v.-immunoglobulins: new approaches to the treatment of Guillain-Barre' syndrome.

We report the results obtained with plasma exchange (PE) in 26 patients (18 males, 8 females) with Guillain-Barré Syndrome (GBS) of maximal severity and also the results with IVGG in 7 patients (3 males, 4 females) where PE could not be employed. A complete clinical and neurophysiological evaluation was carried out. A follow-up was effected at regular intervals for a year. From this study the efficacy of PE in GBS was confirmed and a correlation was found between the amplitude of the muscular potential registered in the acute phase and the prognosis of the illness. PE is not effective for patients with a fulminant course and blocking of conduction. Good results have been obtained with IVGG therapy, which seems to be safe and effective whenever PE is not possible.

[Tolosa-Hunt syndrome. TC findings in a case]. / La sindrome di Tolosa Hunt. Reperti TC in un caso.

The Tolosa Hunt Syndrome is a rare neurological disease. It can be the consequence of a number of processes localized in the cavernous sinus or near to it. The diagnosis has been until now a diagnosis of exclusion, even if the phlebography can give more informations. Recently the possibility of demonstrating the process has been obtained by the high resolution CT scan that can show peculiar findings as in the case that we are describing.

[Clinical and neurophysiologic study of Guillain Barré syndrome: results of therapy with plasmapheresis]. / Studio clinico e neurofisiologico nella sindrome di Guillain Barré: risultati della terapia con plasmaferesi.

The Guillain Barré poliradicoloneuritis shows a high incidence of permanent sequelae and exitus. Recently the plasmapheresis has been applied in the acute stage with a beneficial effect to the rapidity and the degree of the recovery. In the present study the results obtained in a group of patients with Guillain Barré of maximal severity are reported. All the patients have received plasmapheresis (3-5 exchanges) in the first days of the disease. Eventual side effects during the procedure have been evaluated together to the neurophysiological findings. The recovery has been evaluated in the brief and in the long term. From this study the efficacy of the plasmapheresis in Guillain Barré poliradicoloneuritis is confirmed. Furthermore a correlation has been evidenced between the amplitude of the muscular potential (CMAP) registered in the acute phase and the prognosis of the illness.

Myeloid antigen expression in adult acute lymphoblastic leukemia: clinicohematological correlations and prognostic relevance.

Fifty adult patients with acute lymphoblastic leukemia (ALL) were prospectively studied to determine the clinical and hematological relevance of surface immunophenotypes. Before treatment, blast cells were assayed for reactivity to monoclonal antibodies to B-cell, T-cell, and myeloid (My) antigens. My antigens (CD13, CD33, and VIM2, singly or in combination) were demonstrated in 16 cases (32%) along with lymphoid specificities. Bone marrow and peripheral blood stains were classified according to French-American-British (FAB) Cooperative Group criteria and evaluated for myelodysplastic changes and azurophilic granules. Mean age of My+ patients was significantly higher. Furthermore, a greater number of My+ cases showed azurophilic cytoplasmic granules and acid ANAE positivity. FAB subtypes and myelodysplastic features did not significantly differ in the two groups analyzed, but patients with myelodysplastic abnormalities represented a significantly older age group. Response to treatment was comparable in My+ and My- cases, in terms of either complete remission rate or median survival duration.

Memory performances after anterior communicating artery aneurysm surgery.

10 patients operated on for anterior communicating artery aneurysm (ACoA) underwent to neuropsychological examination one year after surgery, in order to detect eventual behavioural and/or amnestic disorders, already described by others in a wide percentage of such patients. Even if after accurate neurological examination the therapeutic results were defined as "good", 60% circa of our patients present amnestic and/or behavioural disturbances. Possible correlations between neuropsychological tests finding and intraoperative occurrences (early aneurysm rupture, gyrectomy etc.) are considered and discussed.

An analysis of morphological, cytochemical, immunological and clinical features of adult T cell acute lymphoblastic leukemia.

Morphological, cytochemical, immunological and clinical features of 19 adult patients with T cell acute lymphoblastic leukemia (T-ALL) were investigated at the time of diagnosis and were compared with those of 34 adult patients affected by B cell acute lymphoblastic leukemia (B-ALL). Immunophenotypic studies employing a wide panel of monoclonal antibodies (mAbs) revealed a heterogeneous pattern of antigen expression in the five T-ALL groups that were identified on the basis of blast T cell differentiation levels. PAS (periodic acid Schiff) negativity and focal AP (acid phosphatase) positivity, as well as white blood cell count and serum lactic dehydrogenase levels, were significantly related to T-ALL when compared with B-ALL. On the contrary, no statistically significant difference was demonstrated in the clinical outcome.

Cerebral blood flow in thalamic aphasia.

A 59-year-old man is reported, who became aphasic after left thalamic infarction, shown by CT. His speech was fluent, with reduced voice volume, impaired auditory and reading comprehension, verbal paraphasias but intact repetition skills. A single photon emission computed tomography (SPECT) scan to measure regional cerebral flow (rCBF) showed a reduction of flow in the parietotemporal areas of the left hemisphere. It is suggested that thalamic aphasia could result from structural subcortical damage with a homolateral functional cortical deficit leading to the specific aphasic disturbance.

[The alexias]. / Le alessie.

The authors propose a review about Alexias according to the latest theories. Anatomoclinical and linguistic classification of alexia are reported. Some hypotheses about reading processes, according to linguistic classification, are discussed.