Association between dysarthria and cognitive impairment in ALS: A prospective study.

Several studies have demonstrated impaired cognition in amyotrophic lateral sclerosis (ALS) patients, but it has been difficult to identify risk factors for this impairment. An association between cognitive changes and bulbar site of onset or dysarthria has been suggested, but the findings are variable. We tested for both associations in a large cohort of ALS patients. At the time of diagnosis of sporadic ALS, all patients (n=355) in this prospective study underwent comprehensive neuropsychological testing. In addition, a subset of 175 patients underwent a detailed assessment of dysarthria, which was quantified using the Appel ALS Score (AALSS). ALS patients with bulbar site of onset performed significantly worse than limb onset patients on a few timed ((VSAT-time, p<0.05), (Stroop Color, p<0.05), (Stroop Word, p<0.05)) tests of frontal lobe functions, but the significance could not be replicated when motor impairment was accommodated into the tests ((VSAT-errors, p=0.73), (Stroop interference, p=0.08)). ALS patients with dysarthria performed significantly worse than non-dysarthrics on multiple timed ((BD, p<0.05), (VSAT-time, p<0.05), (Stroop Color, p<0.05), (Stroop Word, p<0.05), (Trails A, p<0.05), (Trails B, p<0.05)) neuropsychological tests, and the significance was maintained when motor impairment was accommodated into one of these tests (Stroop interference, p<0.05). Additionally, dysarthrics performed significantly worse on two untimed measures of cognition ((Similarities, p<0.05), (Rey Copy, p<0.05)). Cognitive functioning in ALS does not associate with the site of onset and has a moderate association with dysarthria.

Familial occurrence of complex regional pain syndrome.

BACKGROUND: The etiology of complex regional pain syndrome (CRPS) is unknown. Different environmental and genetic factors have been postulated to contribute to CRPS. METHODS: We reviewed the clinical data from a cohort of 69 patients with CRPS. Four families were identified with two or more members affected with CRPS yielding a total of nine patients. Six more patients reported the presence of pain symptoms in their family members, however; this could not be clinically confirmed. RESULTS: The case histories of the nine individuals with 'familial' CRPS suggested a younger age at onset and more frequent history of migraine versus the non-familial patients. A pattern of inheritance could not be ascertained. CONCLUSION: This data supports the hypothesis that CRPS can be familial and hence may have a genetic basis in some families. Larger studies will be needed to ascertain clearer patterns of inheritance and to determine whether the clinical features of 'familial' CRPS are the same as the sporadic form.

Ketamine treatment for intractable pain in a patient with severe refractory complex regional pain syndrome: a case report.

In this case report, we describe the effect of ketamine infusion in a case of severe refractory complex regional pain syndrome I (CRPS I). The patient was initially diagnosed with CRPS I in her right upper extremity. Over the next 6 years, CRPS was consecutively diagnosed in her thoracic region, left upper extremity, and both lower extremities. The severity of her pain, combined with the extensive areas afflicted by CRPS, caused traumatic emotional problems for this patient. Conventional treatments, including anticonvulsants, bisphosphonates, oral steroids and opioids, topical creams, dorsal column spinal cord stimulation, spinal morphine infusion, sympathetic ganglion block, and sympathectomy, failed to provide long-term relief from pain. An N-methyl-d-aspartate (NMDA) antagonist inhibitor, ketamine, was recently suggested to be effective at resolving intractable pain. The patient was then given several infusions of intravenous ketamine. After the third infusion, the edema, discoloration, and temperature of the affected areas normalized. The patient became completely pain-free. At one-year of follow-up, the patient reported that she has not experienced any pain since the last ketamine infusion. Treatment with intravenous ketamine appeared to be effective in completely resolving intractable pain caused by severe refractory CRPS I. Future research on this treatment is needed.

Lumbar puncture alleviates chorea in a patient with Huntington's disease and normal pressure hydrocephalus.

A 44-year-old African-American male was admitted to our hospital after a suicide attempt. He had depression, poor cognitive function, choreiform movements, difficulty pronouncing words, and difficulty walking. His symptoms had worsened markedly over several months. Chorea lead to genetic testing that confirmed a diagnosis of Huntington Disease (HD). A CT scan of the head showed wider ventricles than is typical of HD. The head CT and gait change suggested normal pressure hydrocephalus (NPH). Lumbar puncture (LP) led to improved neuropsychologic test scores and walking thereby supporting the diagnosis of NPH. Surprisingly, the LP also led to an 80% improvement of chorea. There are two other reports of an association between HD and NPH. NPH should be considered in HD patients with atypical symptoms, such as the inability to walk or rapid progression, as its treatment may lead to improved cognition, gait, and chorea.