Long-term follow-up of a case of adult-onset Alexander disease presenting with cognitive impairment as the initial symptom

Abstract Aims Takotsubo syndrome (TTS) is an acute and reversible left ventricular dysfunction, whose pathophysiological mechanisms are not completely known. There are evidence suggesting a possible link between neurological disease and TTS. Aim of the study was to evaluate incidence and prognostic value of cognitive neurological disorders among TTS patients. Methods and results 379 consecutive patients were enrolled in a prospective multicentre registry. History, clinical features, echocardiographic parameters, in-hospital complications and long-term Follow-up events of all patients were recorded. Cognitive neurological disorders included Alzheimer disease, old age dementia and cognitive impairment for other causes. Prevalence of cognitive neurological disorders among TTS patients was 5.5% (num = 21). Among this subset of patients 48% (num = 10) had Alzheimer syndrome, 24% (num = 5) old age dementia and 28% (num = 6) cognitive impairment for other causes. Compared to the control group, these patients were older (81 ± 5 vs. 71 ± 12, P = 0.01) and predominantly men (24% vs. 9%, P = 0.01). No differences in term of cardiovascular risk factors and left ventricular ejection fraction at admission and discharge were found among the two groups. TTS patients with cognitive neurological disorders experienced higher rate of in-hospital complications (62% vs. 28%, P = 0.01), that were mainly driven by higher rate of pulmonary oedema (14% vs. 9%, P = 0.01), cardiogenic shock (29% vs. 8%, P = 0.01), death (24% vs. 4% P = 0.01), ischaemic stroke (10% vs. 4%, P = 0.01), and left ventricular thrombi (10% vs. 3%, P = 0.01). At long-term follow-up patients with cognitive neurological disorders when compared to those without, experienced higher rate of mayor cardiovascular events (48% vs. 16%, P = 0.01), cardiovascular re-hospitalization (14% vs. 10%, P = 0.01) and death (43% vs. 9%, P = 0.01). Conclusions TTS patients with cognitive neurological disorders had an increased risk of in and out of hospital mayor cardiac adverse events and mortality at short and long-term follow-up.

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Evolution of Cognitive Deficits and Conversion to Dementia in Patients with Mild Cognitive Impairment: A Very-Long-Term Follow-Up Study

Dementia and Geriatric Cognitive Disorders ◽

10.1159/000092534 ◽

2006 ◽

Vol 21 (5-6) ◽

pp. 380-391 ◽

Cited By ~ 68

Author(s):

John R. Hodges ◽

Sharon Erzinçlioğlu ◽

Karalyn Patterson

Keyword(s):

Cognitive Impairment ◽

Mild Cognitive Impairment ◽

Cognitive Deficits ◽

Follow Up Study ◽

Long Term Follow Up

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Natural History of Adult-Onset Laryngeal Papillomatosis following Multiple Cidofovir Injections

Annals of Otology Rhinology & Laryngology ◽

10.1177/000348940611500303 ◽

2006 ◽

Vol 115 (3) ◽

pp. 175-181 ◽

Cited By ~ 22

Author(s):

Ana Nusa Naiman ◽

Darius Abedipour ◽

Sonia Ayari ◽

Elizabeth Fresnel ◽

Bruno Coulombeau ◽

...

Keyword(s):

Complete Remission ◽

Surgical Excision ◽

Intralesional Injection ◽

Adult Onset ◽

Laryngeal Papillomatosis ◽

Long Term Follow Up ◽

History Of ◽

A Prospective Study

Objectives: A prospective study was performed to assess the intermediate and long-term efficacy of intralesional cidofovir therapy associated with surgical excision in laryngeal papillomatosis in adults. Methods: Endoscopy with intralesional injection of cidofovir 5 mg/mL was performed 3 times at 4-week intervals. The concentration was later increased to 7.5 mg/mL and the interval between injections shortened to 2 weeks. Further treatment was performed at 3 or 6 months, depending on the evolution of the papillomas. After complete remission, the treatment was stopped and the patients were reviewed every 6 months. Results: Nineteen patients completed the protocol, with a mean of 4.5 injections each. Complete remission was obtained in 17 cases (89%) after a mean of 3.8 procedures. Remission was stable after a mean follow-up of 24 months (range, 8 to 57 months). With higher cidofovir concentrations at shorter intervals, patients needed fewer injections to achieve remission (mean, 2.1 versus 4.7 injections). Conclusions: The effectiveness of intralesional cidofovir therapy in adult-onset recurrent respiratory papillomatosis was impressive. Once obtained, complete remission was stable on intermediate or long-term follow-up. The concentration and the interval between injections seemed to influence the number of injections necessary to achieve remission.

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Extensive Limb Lengthening for Achondroplasia and Hypochondroplasia

10.20944/preprints202105.0268.v1 ◽

2021 ◽

Author(s):

Dror Paley

Keyword(s):

Skeletal Maturity ◽

Limb Lengthening ◽

Adult Onset ◽

Juvenile Onset ◽

Normal Height ◽

Height Range ◽

Long Term Follow Up ◽

Males And Females

Extensive limb lengthening (ELL) was completed in 75 patients: 66 achondroplasia and 9 hypochondroplasia. The average lengthening was 27cm for achondroplasia (12-40cm) and 17cm for hypochondroplasia (range 10-25cm). There were 48 females and 27 males. Lengthening was done either by 2-segment (14 patients; both tibias and/or both femurs) or by serial 4-segment lengthenings (64 patients; both femurs and tibias same time). Most patients also had bilateral humeral lengthening. Lengthenings were either juvenile-onset (31), adolescent-onset (38) or adult-onset (6). The average age at final follow-up was 26 years old (range 17-43 years). There were few permanent sequelae of complications. The most serious was one paraparesis. All patients returned to activities of normal living and only one was made worse by the surgery (paraparesis). This is the first study to show that ELL can lead to increase of height into the normal height range. Previous studies showed mean increases of height of up to 20cm, while this study consistently showed an average increase of 30 cm (range 15-40cm) for juvenile-onset and increase of 26cm (range 15-30cm) for adolescent-onset. This results in lower normal height at skeletal maturity for males and females. The adult-onset had a mean increase of 16.8 (range 12-22cm). This long-term follow-up study shows ELL can be done safely even with large lengthenings and that 4-segment lengthening may offer advantages over 2-segment lengthening. While the majority of cases were performed using external fixation, implantable limb lengthening promises to be an excellent alternative and perhaps an improvement.

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