Brainstem auditory pathway maturation in term neonates with congenital cytomegalovirus infection: a cohort study.

Congenital cytomegalovirus infection (cCMVi) is a leading cause of sensorineural hearing loss (SNHL) and developmental delay. Brainstem auditory evoked potentials (BAEPs) recording allows assessment of central auditory pathway maturation in neonates. We aimed to characterize the effect of cCMVi on the maturation of the brainstem auditory pathway in term neonates.

Biallelic DMXL2 mutations impair autophagy and cause Ohtahara syndrome with progressive course.

Ohtahara syndrome, early infantile epileptic encephalopathy with a suppression burst EEG pattern, is an aetiologically heterogeneous condition starting in the first weeks or months of life with intractable seizures and profound developmental disability. Using whole exome sequencing, we identified biallelic DMXL2 mutations in three sibling pairs with Ohtahara syndrome, belonging to three unrelated families. Siblings in Family 1 were compound heterozygous for the c.

Diagnostic value of Hoover sign and motor-evoked potentials in acute somatoform unilateral weakness and sensory impairment mimicking vascular stroke.

Acute unilateral weakness along with sensory impairment is commonly caused by obstruction of major cortical arteries in either adults or children. A somatoform presentation mimicking acute vascular stroke is very rare, especially in the pediatric age group. Here we report three adolescents presenting with acute unilateral weakness and sensory impairment along with diminished tendon reflexes who were suspected to have an acute stroke but who had developed a somatoform psychogenic disorder.

Motor cortex disinhibition in normal-pressure hydrocephalus.

Object: Previous studies have shown a close association between frontal lobe dysfunction and gait disturbance in idiopathic normal-pressure hydrocephalus (iNPH). A possible mechanism linking these impairments could be a modulation of corticospinal excitability. The aim of this study was 2-fold: 1) to determine whether iNPH affects corticospinal excitability; and 2) to evaluate changes in corticospinal excitability following ventricular shunt placement in relation to clinical outcome.

[Acute hemiplegia and hemianesthesia together with decreased tendon reflexes mimicking acute stroke representing a conversion disorder].

Acute hemiplegia and hemianesthesia is commonly caused by obstruction of major cortical arteries. Such a presentation secondary to a conversion reaction is very rare, especially in the pediatric age group. The authors report an adolescent presenting with acute complete left-sided hemiplegia and sensory loss together with decreased tendon reflexes mimicking an acute arterial stroke.

Effect of electroconvulsive therapy on cortical excitability in patients with major depression: a transcranial magnetic stimulation study.

Objective: The antidepressant action of electro-convulsive therapy (ECT) and repetitive transcranial magnetic stimulation (rTMS) may be related to their ability to modulate cortical excitability. The aim of this study was to investigate changes in cortical excitability following ECT in patients with major depression (MD) and to compare therapeutic efficacy of ECT combined with rTMS to that of ECT alone.

Methods: Twenty-two patients with MD were assigned to receive ECT and right prefrontal 1 Hz rTMS (n=12) or ECT with sham rTMS (n=10).

Evidence for cortical hyperexcitability of the affected limb representation area in CRPS: a psychophysical and transcranial magnetic stimulation study.

Functional alterations in noxious, sensory and motor circuits within the central nervous system may play an important role in the pathophysiology of complex regional pain syndrome (CRPS). The aim of the present study was to search for further evidence of hyperexcitability in the hemisphere contralateral to the affected limb in patients with CRPS by employing both psychophysical and transcranial magnetic stimulation (TMS) methods. Twelve patients with CRPS type I, confined to the distal part of a limb (six in an upper-limb and six in a lower-limb), were enrolled in the study.

The value of motor and somatosensory evoked potentials in evaluation of cervical myelopathy in the presence of peripheral neuropathy.

Study Design: Patterns and rates of motor-evoked potential (MEP) and somatosensory-evoked potential (SEP) abnormalities were evaluated in 9 patients with combined cervical cord compression and diabetic neuropathy and 15 patients with asymptomatic cervical cord compression. The results were compared with those of 8 patients with pure cervical myelopathy and 7 patients with pure diabetic neuropathy.

Objective: To assess the efficacy of MEPs and SEPs in the evaluation of cervical myelopathy in the presence of peripheral neuropathy.

Sound localization in patients with cochlear implant--preliminary results.

Objectives: To evaluate sound localization ability in patients with unilateral cochlear implant, who do not wear a hearing aid on the contralateral ear, and to try to improve this ability by training.

Setting: Tertiary academic referral center.

Methods: In the initial test, patients were exposed to sound stimuli from different directions and were asked to localize them.