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Biomedical subjects

Anamarli Nucci

Publications and source records attributed to Anamarli Nucci.

7 recordsLinked to original sources

Brain tumor-like lesion in Behçet disease.

We report a patient with longstanding Behçet disease who presented sudden onset of headache and facial paresis. The magnetic resonance imaging (MRI) showed a mass in the right thalamus, extending to the lentiform nucleus, subthalamic area, right cerebral peduncle and deep subcortical white matter. Stereotactic brain biopsy disclosed gliosis with no signs of malignancy. The diagnosis of a pseudotumoral form of neuro-Behçet disease was done and she was treated with pulse methylpredinisolone and intravenous cyclophosphamide. After 8 weeks she had improved and a new MRI showed disappearance of the tumor-like lesion. The differential diagnosis, especially with central nervous system tumor is emphasized.

Adult↗

Guillain-Barré syndrome in the elderly: clinical, electrophysiological, therapeutic and outcome features.

UNLABELLED: There are few papers devoted to geriatric Guillain-Barré (GBS) and many related issues remain unanswered. OBJECTIVE: To describe clinical, electrophysiological and therapeutic features in this age. METHOD: Clinico-epidemiological data and therapy of GBS patients older than 60 years were reviewed. Hughes scores were used to quantify neurological deficit and define outcome. RESULTS: Among 18 patients (mean age 64.8 years), 9 had evident prodrome and 80% noticed initially sensory-motor deficit. Demyelinating GBS was found in 8 and axonal in 6 subjects. There was one Miller-Fisher and 3 unclassified cases. Plasmapheresis (PFX) was single therapy in 12 patients and intravenous immunoglobulin (IVIg) in 2. Disability scores just before therapy were similar in both groups, so as short and long term outcome. CONCLUSION: Axonal GBS seems to be more frequent in the elderly and this may have prognostic implications. PFX and IVIg were suitable options, but complications were noticed with PFX. Prospective studies are needed to better understand and manage GBS in the elderly.

Age Factors↗

Multi-minicore disease revisited.

Multi-minicore disease (MmD) is an infrequent congenital myopathy, defined by structural changes in optic and electron microscopy, namely, multiple small areas lacking oxidative enzyme activity and focal disorganization of contractile proteins involving at most a few sarcomeres. The classical form of the disease manifests as more or less severe hypotonia and generalized weakness with predominance in axial and proximal limb muscles. Clinical variants also exist. Usually MmD is inherited as an autosomal recessive trait. Genetic heterogeneity is recognized and up to now mutations in the genes of RYR1 and SEPN1 have been detected. We record three unrelated cases of MmD. Case 1, with the classical benign form, was followed-up for 15 years. Case 2, presenting pharyngolaryngeal involvement and severe delay of head control, improved gradually, until independent gait was acquired at age of six years. A moderate restriction of daily life activities remains. Case 3, of antenatal-onset, was expressed by arthrogryposis of hands, predominance of scapular girdle deficit and a stable course after ten years on physiotherapy. All cases were selected by the characteristic morphological abnormalities in biceps brachii samples, including electron microscopy. Emphasis is given to case 2 due to type 1 fiber uniformity and mild endomysial fibrosis, posing a difficult differential diagnosis with congenital muscular dystrophy were it not for the significant number of multi-minicores.

Adolescent↗

Plasmapheresis in the treatment of myasthenia gravis: retrospective study of 26 patients.

We analyzed the experience of Unicamp Clinical Hospital with plasma exchange (PE) therapy in myasthenia gravis (MG). About 17.8 % of a totality of MG patients had PE performed: 26 cases, 19 women and seven men. The mean age-onset of MG was 28 years, extremes 11 and 69. Minimum deficit observed in the group was graded IIb (O & G) or IIIa (MGFA scale). One patient had prethymectomy PE. In seven the procedures were performed due to myasthenic crisis and in 18 patients due to severe myasthenic symptoms or exacerbation of previous motor deficit. Two patients were also submitted to chronic PE considering refractoriness to other treatments. Twenty-six patients had 44 cycles of PE and 171 sessions. The mean number of sessions was 3.9 (SD +/- 1.4) each cycle; median 5, extremes 2 and 6. The mean time by session was 106,5 minutes (SD +/- 35.2); median 100.5 (extremes of 55 and 215). The mean volume of plasma exchanged in each session was 2396 ml (SD +/- 561); median 2225 (extremes 1512 and 4500). Side effects occurred: reversible hypotension (seven cases), mild tremor or paresthesias (seven cases). Infection and mortality rates due to PE were zero. All patients had immediate benefit of each PE cycle and usually they also received prednisone or other immunosuppressors. Good acceptance of the procedure was observed in 80.7% of patients.

Adolescent↗

Meningoradiculitis due to Cryptococcus neofermans in an immunocompetent patient.

Meningoradiculitis refers to combined involvement of meninges and nerve roots. The most frequent location is the lumbosacral region. Etiology is diverse, including inflammatory, infectious and neoplastic disorders. Meningoradiculitis is a rare form of involvement in cryptococcal infection. We describe a case of subacute lower limbs flaccid paresis diagnosed as lumbosacral meningoradiculitis in view of cerebrospinal fluid (CSF) inflammatory changes and typical enhancement on MRI of lumbar spine. Cryptococcus neoformans was isolated from CSF. Extensive screening yielded no immunodeficiencies.

Adolescent↗

Dorsal ulnar cutaneous nerve conduction: reference values.

We investigated the reference values of the dorsal ulnar cutaneous nerve (DUC) sensory nerve conduction (SNC) in 66 healthy individuals. Measurements were processed using stimulating electrodes positioned between the ulnar bone and the flexor carpi ulnaris muscle, 11-13 cm proximal to the active electrode recording. Superficial recording electrodes were placed on the fourth intermetacarpal space. The mean sensory conduction velocity (SCV) in males was 63.7 - 0.16 x age +/- 3.36 m/s and in females was 57.7 +/- 3.37 m/s. The mean sensory nerve action potential (SNAP) amplitude in males was 19.5 +/- 10.7 microV and in females was 24.6 +/- 5.8 microV. The mean SNAP duration was 0.96 +/- 0.13 ms. No significant differences regarding the DUC-SCV, distal latency, and SNAP duration or amplitude were found between both sides of the same subject. The amplitude of the SNAP was higher in females than males. The effects of age on DUC-SCV were distinct for each gender.

Adolescent↗