Epstein-Barr virus meningoencephalitis in a immunocompetent child

Introduction: Meningoencephalitis caused by the Epstein-Barr virus (EBV) is a rare neurological entity, most often related to the latent reactivation of thevirus associated with immunosuppression. The clinic varies between encephalitis, meningitis, cerebellitis, neuritis and transverse myelitis. The prognosis is good, with 85% of patients returning to baseline levels. Case report: We attend to a 11-month-old male patient, proper neuropsychomotor development, with reports of fever and odynophagia, without improvement with antibiotic therapy. He had a focal epileptic seizure. Upon admission, he presented a new similar event, with control after use of diazepam and intravenous phenobarbital. At the neurological examination carried out after improving the level of consciousness, the patient was alert. He presented axial hypotonia, dystonic posture in flexion of the right upper limb, with preserved strength. He showed no meningeal signs. The cerebrospinal fluid revealed 61 mg/dL of glucose, 57 mg/dL of proteins and 65/mm³ of leukocytes (90% of lymphocytes). Acyclovir, ceftriaxone, and dexamethasone were started, with improvement. Magnetic resonance imaging revealed: FLAIR hyper signal injury, with diffusion restriction, in the left caudate nucleus, left lentiform and ipsilateral anterior midbrain region. Electroencephalogram with diffuse attenuation of baseline electrical activity. The main diagnostic hypothesis was meningoencephalitis secondary to EBV infection, with confirmatory PCR-DNA. Conclusion: The report illustrates the need for attention to a rare infectious etiology in atypical presentation of meningoencephalitis.

Treatment of Dystonia Using Trihexyphenidyl in Costello Syndrome

Costello syndrome (CS), a rare syndrome with multisystemic involvement inherited as a dominant trait, is characterized by developmental delay, coarse facial appearance, cardiac defects including hypertrophic cardiomyopathy, skin abnormalities, brain complications, and a predisposition to certain malignancies. The musculoskeletal system is particularly affected in CS, with peculiar orthopedic anomalies that impact posture and gait. Dystonia has been recently documented to contribute to abnormal postures and musculoskeletal anomalies characterizing CS, suggesting the possible use of pharmacological treatments to treat these complications. We report the case of a child affected by CS displaying a particularly severe musculoskeletal involvement with dystonic posture especially in the arms and legs. The Movement Disorder-Childhood Rating Scale (MD-CRS) and a gait analysis were used to assess clinical patterns of hyperkinetic movement disorder and dystonia. The child was further treated with trihexyphenidyl for six months with a final dosage of 14 mg. MD-CRS and gait analysis assessments provided evidence for a significant improvement of posture and the related musculoskeletal problems with no side effects. Our preliminary study report provides first evidence that pharmacological anti-dystonia treatment significantly improves movement and posture disorders in patients with CS. Further studies enrolling larger cohorts of patients should be performed to validate these preliminary observations.

Tricks that relieve camptocormia during gait in Parkinson’s disease patients

Introduction. Camptocormia is an extreme flexion of the toracolum­bar spine that exacerbates by walking and is relieved in the supine position. It is described with increasing frequency in association with Parkinson disease (PD). Its pathogenesis remains unclear. One of the hypotheses proposed defends that when associated with PD probably it is a focal action dystonia of the paraspinal muscles. Dystonia may be accompanied of a “geste antagoniste”. It is a kind of trick that en­ables the patient to alleviate dystonic posture. We present two cases in which the patients use sensory tricks to alleviate their posture during gait. Case Reports. Patient 1: A 74-year–old woman, with a 2-year of PD. Clinical examination disclosed parkinsonian signs and camp­tocormia. She was able to partially overcome it by pushing her hands against her thighs. Patient 2: A 80-year-old woman, with a 1-year probable PD. Neurological examination revealed postural instability, freezing gait and camptocormia that partially remits when she pushes a bar at the level of her waist. Conclusions. The particularity of these cases is that patients are able to improve camptocormia during gait by specific tricks. This clinical improvement supports the hypothesis that camptocormia in PD is a focal dystonia of the paraspinal muscles.

Partial myotomy/myectomy of the trapezius muscle with an asleep-awake-asleep anesthetic technique for treatment of cervical dystonia

✓ The authors describe a technique for performing partial sectioning and myectomy of the trapezius muscle in patients with severe cervical dystonia that is unresponsive to conservative treatment. Asleep-awake-asleep anesthesia allows intraoperative control of the sectioning procedure to avoid causing postoperative weakness of arm elevation above the horizontal plane. The procedure has been performed successfully in three patients. In all cases the dystonic posture of the shoulder and local pain were improved postoperatively. There were no new deficits. This technique can be used as an adjunct to other peripheral surgical procedures in patients with marked laterocollis and dystonic elevation and anteversion of the shoulder.

Dopa-sensitive progressive dystonia of childhood with diurnal fluctuations of symptoms: a case report

Progressive dystonia with diurnal fluctuations sensitive to levodopa, also known as Segawa's disease, is a rare form of autosomal dominant extrapyramidal disease in the pediatric age group. The dystonic and Parkinson-like symptoms are the main clinical features of the disease and, characteristically but not in all cases, show a diurnal variation. They are absent or present to a lesser extent in the morning, worsening during the day. Treatment with small doses of levodopa results in remission or marked improvement of the symptomatology. We present the case of a 11 years old female patient that developed a dystonic posture in her feet that led her to a tip-toe walking pattern, since the age of 2. Diurnal fluctuations of the symptomatology were noticed by her mother. At 7 years of age she developed a left deviation of the head and an abnormal flexor posture of the left arm. In the next years the symptoms progressed and the fluctuations became less evident. At the age of 10, they were present soon after she woke up in the morning. The neurological examination disclosed a dystonic posturing of the head and left arm, a generalized rigidity of the extremities and a palpebral tremor. Laboratory examinations, including copper and ceruloplasmin, and neuro-imaging studies were negative. She was started on levodopa 150 mg/day with prompt disappearance of the symptomatology. After one-year follow-up she is symptom-free with only 100 mg/day of levodopa. No adverse effect was observed so far.