Rapidly progressive aphasic dementia with motor neuron disease: a distinctive clinical entity.

The association of motor neuron disease (MND) with rapidly progressive aphasic dementia has been recognized as a distinct clinical syndrome within the group of frontotemporal dementias (FTDs). Although the clinical and neuropsychological features of this syndrome have been defined, a small number of post-mortem studies have been published with heterogeneous neuropathological findings. We performed cognitive, neuro-imaging and neuropathological studies on a 71-year-old male with rapidly progressive aphasic dementia and MND. We initially found a selective non-fluent aphasia associated with hypoperfusion of the left frontotemporal cortex. Proton magnetic resonance spectroscopy revealed an asymmetric change of brain metabolites, with greater changes in the left temporal lobe. The bulbar manifestations of MND occurred over the following 6 months, and the patient died of bronchopneumonia. The neuropathological examination revealed loss of neurons in the hypoglossal nucleus and anterior horns of the cervical spinal cord with microvacuolation and dot-like ubiquitin-positive deposits in the frontoparietotemporal cortex, but no changes suggestive of Alzheimer's, Pick's or Lewy body disease. These findings support the conclusion that MND with rapidly progressive aphasic dementia is a distinctive clinical entity within the group of FTD-MND.

An Italian survey of traumatic spinal cord injury. The Gruppo Italiano Studio Epidemiologico Mielolesioni study.

OBJECTIVE: To describe the etiology, clinical presentation, complications, outcome indicators, and links between emergency and acute intervention and rehabilitation of patients with traumatic spinal cord injury (SCI). DESIGN: Multicenter prospective study involving patients with SCI discharged, after rehabilitative care, between February 1, 1997, and January 31, 1999. SETTING: Thirty-two Italian hospitals involved in SCI rehabilitation. PARTICIPANTS: Six hundred eighty-four patients with traumatic SCI on their first admission to a rehabilitation center. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Neurologic improvement (NI), bladder autonomy, feelings of dependency, and destination were evaluated on discharge. Pressure ulcers on admission, time from injury to admission, and length of stay (LOS) were considered as indirect measures of the effectiveness of the health system. RESULTS: Traumatic etiology had a male-to-female ratio of 4:1 (548:136). Collision on the road was the main cause of traumatic injury (53.8%). Mean time from injury to admission was 36.8 days; 126 patients (18%) were admitted within the first week after injury. Mean LOS was 135.5 days. In 184 patients (26.9%), a pressure ulcer was present on admission. On discharge, NI was recorded in 179 patients (26.2%), whereas 446 (65%) and 418 (61%) had bladder and bowel autonomy, respectively, and 560 (81.9%) returned home. In the multivariate analysis, independent variables predicting poor outcome (NI, feelings of dependency, sphincter autonomy, discharge to home, LOS) were related both to the lesion (completeness, cervical involvement) and to the indicators of health service organization (time from injury to admission, complications on admission and during stay). CONCLUSIONS: Our focus on the etiology of traumatic SCI showed that efforts should be made to prevent collisions on the road. Our study also highlights problems in the comprehensive management of patients with SCI in Italy. Better organization could help reduce the time from injury to admission, the number of complications on admission, and LOS, and it could help improve rehabilitation outcome.