Anita M. Devlin

Coma Blisters in 2 Children on Anticonvulsant Medication

Blister formation and eccrine sweat gland necrosis have been recognized to occur in states of impaired consciousness and were first reported following barbiturate intoxication. Their etiology is complex and cannot simply be explained by pressure effects. Now that barbiturates are less frequently used, clinicians are likely to be less aware of the phenomenon of coma blister formation; however, newer drugs have also been associated with the occurrence of coma blisters. We describe 2 new associations of coma blisters and anticonvulsants in children. In the first child, blisters recurred on multiple occasions along with obtundation and edema. Our aims are to alert clinicians to the occurrence of coma blisters in children sedated on anticonvulsant medications and to report the new finding of recurrent coma blisters.

Suspected pheochromocytoma in a patient with Guillain-Barré syndrome.

Autonomic instability is well recognized in Guillain-Barré syndrome (GBS), particularly in the acute inflammatory demyelinating polyneuropathy subtype. Hypertension occurs in up to two-thirds of children with GBS but is rarely the main presenting feature. We describe a teenager who presented with tachycardia, dizziness, flushing, and significant hypertension as well as ascending limb weakness and sensory disturbance with areflexia. Because the predominant initial concern was hypertension, she was referred to pediatric nephrology and appropriate investigations for hypertension were conducted. Her neurologic findings prompted a neurology referral, and a diagnosis of GBS was made. The investigations for hypertension subsequently revealed increased urinary normetadrenaline levels in a range consistent with pheochromocytoma, prompting the question of dual pathology. Both autonomic symptoms and urinary metadrenaline levels subsided with GBS resolution, and further investigations excluded the diagnosis of pheochromocytoma. Our case highlights that significant dysautonomia can occur in children with GBS, with hypertension being a prominent early feature. Recognition that urinary metadrenalines can increase to levels seen in pheochromocytoma is important in avoiding diagnostic confusion.

Teaching NeuroImages: alternating ptosis and Marcus Gunn jaw-winking phenomenon with PHOX2B mutation.

A 2-month-old baby presented with variable right-sided ptosis (without fatigability or maternal myasthenia) and recurrent unexplained central apneas since birth. On sucking, there was a right Marcus Gunn phenomenon with temporary left-sided ptosis (figure; video on the Neurology ® Web site at [www.

Spotting the wolf in sheep's clothing

Jane, a 13-year-old girl, had a 4-week history of abnormal left limb movements and falls. The first problem noted was a high stepping gait on the left which resolved over a few days. Several falls, presumed to be vasovagal faints, ensued and a few days prior to her admission she collapsed after her left leg ‘gave way’. Following this collapse she had persisting left arm weakness and speech difficulties. The general practitioner was concerned she had suffered a stroke and he referred her to hospital for further assessment. The history obtained by the registrar established Jane had been born prematurely at 27 weeks gestation but the pre-, peri- and postnatal course had otherwise been uneventful. She was described as having mild learning difficulties but was in mainstream education with support. In the previous year, she had been seen by the Child and Adolescent Mental Health Service (CAMHS) because of mood swings and some self-harming behaviours. These were attributed to bullying at school and a bereavement reaction to the death of her mother 5 years earlier. With her current presentation, Jane had developed a right hand preference having previously been left hand dominant. She could no longer walk upstairs and had to crawl up while holding on to the banister with her right hand. During the day, her family thought she appeared confused and she would talk for prolonged periods of time, as if to another person, when no-one else was present. She had not slept well for several days and had started to wander about the house, looking for objects which she thought had been lost. There was also a history of fatigue and moderate …

Fifteen-minute consultation: Management of the upper limb in unilateral cerebral palsy

Children with unilateral cerebral palsy (UCP) have complex health, education and social care needs. Delayed gross motor milestones are the most common presenting feature, and much of the early management focuses on gross motor skills and lower limb management. In later childhood, adolescence and adulthood, upper limb function has significant impact on activity, participation and independence. There is clear pathophysiological rationale and emerging clinical evidence that earlier intervention to improve upper limb function is beneficial. Whereas most children with UCP are managed in secondary care, it is recommended that the assessment and delivery of specialist intervention for the upper limb occurs at a regional centre.