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Biomedical subjects

Catherine Lomen-Hoerth

Publications and source records attributed to Catherine Lomen-Hoerth.

8 recordsLinked to original sources
PubMed2007

Dysphagia in patients with frontotemporal lobar dementia.

BACKGROUND: Hyperorality, compulsive eating and aspiration because of food gorging, has been described in patients with frontotemporal lobar dementia (FTLD), but swallowing function in this population has not been reported. OBJECTIVE: To identify the swallowing status in a sample of patients with FTLD. DESIGN: Case series. SETTING: Referral center, ambulatory care. PATIENTS: A consecutive series of referred patients with 3 variants of FTLD were asked to participate. Twenty-one patients were enrolled, including 9 with frontotemporal dementia, 7 with progressive nonfluent aphasia, and 5 with semantic dementia. INTERVENTION: The patients underwent a fiberoptic endoscopic examination of swallowing to assess their ability to swallow liquids and food. MAIN OUTCOME MEASURES: Presence of dysphagia and nature of impaired swallowing. RESULTS: Of the 21 patients, 4 caretakers reported swallowing difficulties. An instrumental examination revealed moderate swallowing abnormalities in 12 of the 21 patients. These abnormalities were not explained by compulsive eating behaviors, but seemed to reflect deficits in cortical and subcortical pathways connecting with the brainstem swallowing center. CONCLUSIONS: When assessed via instrumentation, swallowing abnormalities are found in many patients with FTLD. The appearance of dysphagia signals progression of FTLD to brainstem systems.

Adult↗
PubMed2004

17q-linked frontotemporal dementia-amyotrophic lateral sclerosis without tau mutations with tau and alpha-synuclein inclusions.

BACKGROUND: Frontotemporal dementia (FTD) is a clinically heterogeneous condition that can be associated with clinical manifestations of an extrapyramidal disorder or motor neuron disease. A range of histologic patterns has been described in patients with FTD. The most common familial form of this condition is caused by mutations in the microtubule-associated protein tau gene (MAP tau) and is associated with neuronal or glial tau inclusions. OBJECTIVES: To determine the clinical, anatomic, and pathological features of San Francisco family A and to map the mutation responsible for disease in this family. DESIGN: A systematic clinical, neuropsychologic, neuroimaging, and chromosome segregation analysis of San Francisco family A was performed. A pathological and biochemical assessment of a family member was made. SETTING: Family study. PATIENTS: San Francisco family A, with FTD, variable extrapyramidal symptoms, and prominent motor neuron disease. Afflicted family members do not have a MAP tau coding or splice regulatory sequence mutation, and the MAP tau is genetically excluded. MAIN OUTCOME MEASURES: Comparison of clinical, neuropsychologic, neuroimaging, and linkage findings of San Francisco family A with other familial forms of FTD and amyotrophic lateral sclerosis (ALS). RESULTS: The most probable location for the mutation responsible for this condition is on chromosome arm 17q, distal to the MAP tau. All previously identified susceptibility loci for FTD and ALS are excluded. Autopsy findings from an afflicted family member show distinctive tau and alpha-synuclein inclusions. Another unique feature is that the insoluble tau protein consists predominantly of the 4R/0N isoform. CONCLUSION: The condition affecting members of San Francisco family A is clinically, pathologically, and genetically distinct from previous familial forms of FTD and ALS.

Adult↗
PubMed2004

Characterization of amyotrophic lateral sclerosis and frontotemporal dementia.

Amyotrophic lateral sclerosis (ALS) produces progressive weakness, muscular wasting, and spasticity leading to death from respiratory failure at a median of 3 years after onset. ALS and frontotemporal dementia (FTD) overlap in both familial and sporadic cases of ALS. When both occur in families, the affected members may have only ALS, only FTD, or both. This suggests a relationship in the cause of these disorders. We investigated the frequency of ALS in FTD patients and of FTD in ALS patients and found the overlap to be more common than had previously been reported. We report the features of cognitive impairment and pattern of motor involvement in these ALS-FTD patients, and the degree of overlap in both populations.

Adult↗
PubMed2003

Statistical motor unit number estimation: from theory to practice.

Statistical motor unit number estimation (MUNE) is one of several experimental techniques used to estimate the number of lower motor neurons innervating a given muscle. All are fairly reproducible and have been applied successfully in monitoring neurogenic disease progression. Quantitating the number of lower motor neurons is important, since the compound muscle action potential (CMAP) and strength may not change as rapidly over time due to the confounding effect of reinnervation. MUNE techniques differ in the way they obtain samples of surface-recorded motor unit potentials (SMUP). Statistical MUNE is based on Poisson statistics, uses surface stimulation, and is useful in testing distal, superficial nerves. This review focuses on the theory behind the development of the technique, critiques the publications resulting from applying the technique in control and disease subjects, and discusses the future developments needed for clinical utility.

Action Potentials↗
PubMed2002

The overlap of amyotrophic lateral sclerosis and frontotemporal dementia.

Patients with frontotemporal dementia (FTD) with no known diagnosis of ALS or family history of ALS were clinically and electrophysiologically assessed for the presence of ALS. Of 36 patients studied, five met criteria for a definite diagnosis of ALS and two had EMG findings suggestive of denervation in one limb. An additional five patients had prominent fasciculations and six other patients had trouble swallowing but all had normal results on EMG studies. One of the patients with fasciculations and a normal EMG study progressed to definite ALS over the course of 1 year.

Adult↗