Unilateral cerebellar and brain stem hypoplasia in a child with a postnatal diagnosis of dissecting aneurysm in basilar artery.

Cerebellum is highly vulnerable in the prenatal period. Increasing experience with fetal imaging studies has demonstrated that unilateral cerebellar hypoplasia (UCH) is mainly prenatally acquired, representing disruption rather than a true malformation. Here, we report the case of a 17-month-old boy presented with a sudden onset of abnormal eye movements, who was diagnosed during routine fetal screening with UCH and brain stem hypoplasia and suffered from cerebral palsy; however, no posterior arterial system pathology was detected on cranial magnetic resonance images at that time. Following this acute event, diagnostic neuroradiological interventions revealed a dissecting aneurysm with a saccular component in midbasilar arterial segment and hypoplastic left posterior cerebral artery, which may support the ischemic disruptive mechanism in the development of prenatally detected UCH in this child. The pathogenetic mechanisms for cerebellar disruption are certainly multifactorial in origin, although ischemic arterial etiologies were often undervalued.

Peripheral facial nerve paralysis after upper third molar extraction.

Peripheral facial nerve paralysis (PFNP) after mandibular interventions has been reported in the literature. In most cases, paralysis begins immediately after the injection of the mandibular anesthesia, and duration of facial weakness is less than 12 hours. However, there are few documented cases of PFNP after maxillary dental or surgical procedures. A variety of mechanisms have been associated to PFNP, including viral reactivation, demyelination, edema, vasospasm, and trauma. The purpose of this presentation was to report a rare case of facial paralysis that occurred after an upper third molar extraction. The cause of the PFNP and the importance of the multidisciplinary approach in the management are emphasized.

Guillain-Barré Syndrome and Its Variants: Clinical Course and Prognostic Factors.

INTRODUCTION: We aimed to analyze the frequency, clinical characteristics, medical treatment options and final functional status of Guillain-Barré syndrome (GBS) and its variants in a population from a tertiary hospital setting. METHODS: All medical records of patients with acute inflammatory polyneuropathy between the years of 1998-2013 were retrospectively screened. Demographic, clinical and laboratory information, treatment options and the rate of recovery of the patients were gathered. RESULTS: A total of 183 patients met the study criteria. Subtypes were typical demyelinating form (n=102, 79.1%), acute motor sensory axonal variant (n=11, 8.5%), acute motor axonal variant (n=10, 7.8%), Miller-Fisher syndrome (n=5, 3.9%), and pure sensory subtype (n=1, 0.8%). Remaining patients had the diagnosis of acute-onset chronic inflammatory demyelinating polynuropathy. The data of treatment option were available for 70 patients. Most of the patients received intravenous immunoglobulin (IVIg) treatment or the combination of IVIg and methylprednisolone. One patient died, there was no improvement in eight patients and rest showed improvement with varying degrees. CONCLUSIONS: We did not observe major change of recovery between different treatment options, however, most of the patients using methylprednisolone required IVIg because of inadequate response.

Sensorineural hearing loss and status epilepticus associated with ulcerative colitis: Is there enough evidence to support immune-related mechanisms?

Ulcerative colitis (UC) is characterized by an inflammatory disorder of the gastrointestinal tract. Immune-mediated extraintestinal manifestations of UC have increasingly attracted attention in the literature recently, for which UC is now considered as a systemic disease. Neurologic involvement associated with UC is probably under-reported because of the unawareness of many physicians, although early recognition and treatment are crucial in preventing major morbidity and sequel. In this case report is presented a patient newly diagnosed as UC, who developed both sensorineural hearing loss and intractable status epilepticus that we suggest to have resulted from immune-mediated mechanisms.