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Wolf-Hirschhorn syndrome (WHS) is a multiple congenital anomalies/intellectual disability disorder due to deletion, or loss of material, of the distal portion of the short arm of chromosome 4. Seizures/epilepsy represents one of the main clinical challenges in 4p WHS. Epilepsy is well controlled in most WHS individuals (80%). Phenobarbital has been reported as the most effective drug against tonic-clonic seizures. About 30-40% of the deletions will not be detected by karyotype. Therefore, fluorescence in situ hybridization (FISH) with cosmid probes from the WHS critical region (WHSCR), or comparative genomic hybridization microarray (aCGH) are necessary to confirm the diagnosis. Brain magnetic resonance imaging (MRI) should be performed in all individuals with seizures, since malformation of the cerebral cortex may modify prognosis and management. A waking/sleeping video-EEG-polygraphic study, electrocardiogram [ECG], surface electromyogram [EMG]) is recommended in infancy and childhood, in order to achieve the best characterization of seizures.
The purpose of this study was to describe the outcomes of patients with a severe stroke admitted to a specialized “slow stream” rehabilitation program and to develop a model to predict discharge destination.
Chart review of 196 consecutive non-ambulatory (“lower-band”) stroke patients admitted between 1996-2001, to a specialized in-patient rehabilitation unit designed to accommodate the needs of patients with profound disabilities, and who were considered inappropriate for conventional inpatient rehabilitation programs. Special features of this program included the availability of an independent living unit, therapies tailored to individual tolerance and the opportunity to remain on the unit for an extended period until such time that the patients' rehabilitation potential had been maximized.
Patients were admitted to the unit after a median of 49 days following stroke onset. Their median admission and discharge functional independence measure (FIMTM) scores were 46 and 70, respectively. The improvement in ability to perform self-care tasks was statistically significant (Z= -11.18, p<0.0001). By discharge, 54 patients (28%) were able to ambulate independently (with or without an assistive device), while 142 patients (72%) remained wheelchair dependent. Eighty-five patients (43%) returned to their own home upon rehabilitation discharge, while the remainder were admitted to nursing homes or hospitals closer to the patients' home. Admission FIM score, age, no previous history of stroke and male sex were the variables found to most strongly predict discharge home.
Patients with severe strokes who received individualized care on a highly specialized stroke rehabilitation unit achieved impressive functional outcomes despite a lag of seven weeks post stroke before rehabilitation was initiated. Many patients were no longer wheelchair dependent and almost half returned home. Active rehabilitation should not be limited to “middle-band” stroke patients.
Repeated case series have documented the effectiveness of multidisciplinary in-patient behavioural treatment for conversion disorders. However, in the absence of controlled research, treatment success could be attributed to providing patients with a face-saving opportunity to get better.
The present study contrasts two behavioural treatments to elucidate the factors underlying successful in-patient rehabilitation of this population.
Thirty-nine patients underwent a standard behavioural programme. Using a crossover design, patients who did not improve underwent a strategic-behavioural treatment in which they and their families were told that full recovery constituted proof of an organic aetiology whereas failure to recover was definitive proof of a psychiatric aetiology.
Chart review indicated that the standard behavioural treatment was effective for 8/9 ‘acute’ patients but only for 1/28 ‘chronic’ patients. Of the 21 patients with chronic motor disorder who then under went the strategic-behavioural intervention, 13 were symptom-free at discharge.
The strategic intervention was superior to standard behavioural treatment for patients with chronic motor disorder. Treatment components previously deemed critical for the effectiveness of behavioural treatment may be unnecessary.
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