scholarly journals Long-term Follow-up and Muscle Imaging Findings in Brachio-Cervical Inflammatory Myopathy

2021 ◽  
Vol 8 (4) ◽  
pp. e1016
Author(s):  
Matteo Lucchini ◽  
Sara Bortolani ◽  
Mauro Monforte ◽  
Manuela Papacci ◽  
Enzo Ricci ◽  
...  
Keyword(s):  
Upper Limb ◽  
Age At Onset ◽  
Inflammatory Myopathy ◽  
Diagnostic Delay ◽  
Lower Limbs ◽  
Mri Findings ◽  
Limb Muscles ◽  
Long Term Follow Up

ObjectiveTo report on a cohort of patients diagnosed with brachio-cervical inflammatory myopathy (BCIM), with specific focus on muscle MRI and follow-up data.MethodsClinical, histopathologic, serologic, and pre- and post-treatment MRI findings of patients diagnosed with BCIM were retrospectively evaluated.ResultsSix patients, all females with a mean age at onset of 53 years (range 37–62 years), were identified. Mean diagnostic delay was 17 months, and mean follow-up was 35 months. Most common clinical features encompassed predominant involvement of neck and proximal upper limb muscles, followed by distal upper limb, facial, and bulbar muscle weakness with different severity. Lower limb involvement was rare, although present in severe cases. Muscle biopsies showed a heterogeneous degree of perivascular and endomysial inflammatory changes. Myositis-specific antibodies were absent in all patients, whereas all resulted positive for antinuclear antibodies; half of the patients had anti–acetylcholine receptor antibodies without evidence of muscle fatigability. MRI showed disproportionate involvement of upper girdle and neck muscles compared with lower limbs, with frequent hyperintensities on short-tau inversion recovery sequences. Partial clinical and radiologic improvement with steroid and immunosuppressant therapy was obtained in most patients, especially in proximal upper limb muscles, whereas neck weakness persisted.ConclusionBCIM is an inflammatory myopathy with a peculiar clinical and radiologic presentation and a relatively broad spectrum of severity. Long-term follow-up data suggest that appropriate and early treatment can prevent chronic muscle function impairment. MRI characterization can be helpful in reducing diagnostic and treatment delay with positive consequence on clinical outcome.

Long‐Term Follow‐Up after Finger and Upper‐Limb Replantation: Clinical, Angiologic, and Lymphographic Studies

1998 ◽  
Vol 14 (02) ◽  
pp. 131-136 ◽  
Author(s):  
Claudia Meuli-Simmen ◽  
Marco Canova ◽  
Alfred Bollinger ◽  
Viktor Meyer
Keyword(s):  
Upper Limb ◽  
Long Term Follow Up

scholarly journals A 12-year follow-up of a sample of patients dependent upon heroin

2007 ◽  
Vol 4 (4) ◽  
pp. 94-95 ◽  
Author(s):  
Saima Niaz ◽  
Nadia Arshad ◽  
Mariam Haroon ◽  
Fahd A. Cheema ◽  
Khalid A. Mufti ◽  
...  
Keyword(s):  
Opioid Dependence ◽  
Age At Onset ◽  
Heroin Addiction ◽  
Long Term Follow Up ◽  
History Of ◽  
Employment Rates ◽  
Opium Use ◽  
Significant Mortality

Heroin addiction is a chronic, relapsing and remitting condition. Each year 2–5% of addicts discontinue drug use permanently and 1–2% die, mostly of overdose (Robins, 1993). A study of 129 opiate-addicted patients on a monthly maintenance regimen found that those with a family history of opium use had an earlier age at onset (Chaudhry et al, 1991). Long-term follow-up studies of people who misuse opiates have revealed that opioid dependence appears to run a chronic, relapsing and remitting course with a significant mortality (10–15%) over 10 years (Robson, 1992). Metrebian et al (1998) reported that long-term heroin abstinence was associated with less criminality, psychological distress and morbidity; Hser et al (2001) reported it was associated with higher employment rates.

Headache in progressive facial hemiatrophy (Parry-Romberg syndrome): A paradigmatic case and systematic review of the literature

Cephalalgia ◽  
2021 ◽  
pp. 033310242110434
Author(s):  
Thomas Foiadelli ◽  
Alessandra Rossi ◽  
Chiara Trabatti ◽  
Eugenia Spreafico ◽  
Viola Santi ◽  
...  
Keyword(s):  
Systematic Review ◽  
Age At Onset ◽  
Headache Disorder ◽  
Hemifacial Atrophy ◽  
Long Term Follow Up ◽  
Parry Romberg Syndrome ◽  
Abnormal Brain ◽  
Made In

Background Parry-Romberg syndrome is a neuro-cutaneous disease characterized by progressive hemifacial atrophy. Although common, headache in this population is scarcely reported in the literature. Objective To evaluate the clinical features of headache in pediatric and adult patients with Parry-Romberg syndrome, and to discuss diagnostic and treatment approaches of headache in Parry-Romberg syndrome. Methods We conducted a systematic review in accordance with PRISMA guidelines. We searched the MEDLINE database to identify eligible studies and identified patients with Parry-Romberg syndrome and headache. We further reported a paradigmatic case with a complex headache disorder and described its management and outcome. Results We identified 74 articles, 41 of which were included in the analysis. A total of 52 patients (55.8% female) were included for data analysis. The main age at onset of headache was 20 years (SD 15.2; range 3–56). A diagnosis of migraine was made in 53.9%. Abnormal brain imaging was found in 82.2% of patients. Conclusion Long-term follow-up of patients is required, because headache may develop (and evolve) at any time over the course of the disease. Primary and secondary headaches often co-occur in patients with Parry-Romberg syndrome. Further research into the underlying etiopathogenesis and therapeutic targets would be recommended.

scholarly journals Epileptic Negative Myoclonus as the First and Only Symptom in a Challenging Diagnosis of Benign Epilepsy With Centrotemporal Spikes

2017 ◽  
Vol 4 ◽  
pp. 2329048X1771596 ◽  
Author(s):  
Jing Chen ◽  
Guo Zheng ◽  
Hu Guo ◽  
Xiaopeng Lu ◽  
Chunfeng Wu ◽  
...  
Keyword(s):  
Antiepileptic Drugs ◽  
Upper Limb ◽  
Seizure Type ◽  
Standing Posture ◽  
Long Term Follow Up ◽  
Negative Myoclonus ◽  
Polygraphic Recordings ◽  
Benign Epilepsy

Objective: To investigate the clinical and neurophysiological characteristics of epileptic negative myoclonus as the first and only ictal symptom of benign epilepsy with centrotemporal spikes. Methods: Electrophysiological evaluations included polygraphic recordings with simultaneous video electroencephalogram monitoring and tests performed with patient’s upper limb outstretched in standing posture. Epileptic negative myoclonus manifestations, electrophysiological features, and responses to antiepileptic drugs were analyzed. Results: The authors report 2 patients with benign epilepsy with centrotemporal spikes, who had epileptic negative myoclonus as the first and only seizure type. Video electroencephalogram monitoring results showed that their negative myoclonus seizures were emanating from the contralateral central and the parietal regions. Epileptic negative myoclonus was controlled by administration of valproate and levetiracetam. Conclusion: Epileptic negative myoclonus can be the first and only seizure type of benign epilepsy with centrotemporal spikes, and long-term follow-up monitoring should be the care for the recurrence and/or presence of other types of seizures.

Malignant Melanoma of the Spinal Epidural Space Metastasized from the Enucleated Left Eye after Nine Years without Symptoms: Report of a Case

Neurosurgery ◽  
1985 ◽  
Vol 16 (1) ◽  
pp. 79-82
Author(s):  
Yoshimi Yanai ◽  
Tetsuo Wakao ◽  
Akira Fukamachi ◽  
Hideo Kunimine ◽  
Hirochiyo Wada
Keyword(s):  
Malignant Melanoma ◽  
Epidural Space ◽  
Lower Limbs ◽  
Pertinent Literature ◽  
Long Term Follow Up ◽  
Spinal Epidural

Abstract A case of flaccid paraplegia due to a metastatic spinal epidural melanoma is reported. Symptoms occurred approximately 9 years after the enucleation of the left eye. A solid melanotic mass was removed almost totally. Three years after the operation, the patient had not regained the motility of the lower limbs. The need for long term follow-up and management of patients with such problems is discussed, and the pertinent literature is reviewed. (Neurosurgery 16:79–82, 1985)

scholarly journals Unverricht-Lundborg disease (EPM1) in Finland

Neurology ◽  
2020 ◽  
Vol 95 (23) ◽  
pp. e3117-e3123
Author(s):  
Jussi O.T. Sipilä ◽  
Jelena Hyppönen ◽  
Ville Kytö ◽  
Reetta Kälviäinen
Keyword(s):  
Age At Onset ◽  
Survival Rates ◽  
Functional Independence ◽  
Population Based ◽  
Risk Of Death ◽  
Standard Population ◽  
European Standard Population ◽  
Long Term Follow Up

ObjectiveTo investigate the epidemiology and prognosis of Unverricht-Lundborg disease (EPM1) in a nationwide, population-based setting.MethodsData from multiple registries were combined and analyzed. Clinical data were obtained from medical records. All patients treated for EPM1 in Finland between January 1, 1998, and December 31, 2016 were included.ResultsA total of 135 persons with EPM1 (54% women) were identified and 105 were alive on December 31, 2016 (point prevalence 1.91/100,000 persons). The age-standardized (European Standard Population 2013) prevalence was 1.53/100,000 persons. Annual incidence during the study period was 0.022/100,000 person-years, with a mean age at onset of 9.4 ± 2.3 years (range 7.0–14.6 years, no sex difference). The median age at death (n = 34) was 53.9 years (interquartile range 46.4, 60.3; range 23.2–63.8), with no sex differences. The immediate cause of death was a lower respiratory tract infection in 56% of deaths. The survival rates of the patients were comparable to matched controls up to 40 years of age, but poorer during long-term follow-up (cumulative survival 26.4% vs 78.0%), with a hazard ratio (HR) for death of 4.61. The risk of death decreased with increasing age at onset (HR 0.76 per year, 95% confidence interval 0.65–0.89). In approximately 10% of all cases, the disease progression appeared very mild; some patients retained functional independence for decades.ConclusionsUnverricht-Lundborg disease is rare in Finland but still more common than anywhere else in the world. The disease course appears somewhat more severe than elsewhere, disability mounts early, and death occurs prematurely.

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