Assessment of acute motor deficit in the pediatric emergency room

Abstract

The clinical skills to obtain a complete history and to perform a detailed physical examination are emphasized. An organized, logical, and stepwise diagnostic and therapeutic management is essential to eventually restore patient's well-being and full health.

Guillain‐Barré syndrome in a man presenting with upper extremity monoplegia

“…Pharyngeal-cervical-brachial syndrome (PCB) typically presents as a rapid progression from ptosis, to weakness in the facial, pharyngeal, neck, and arm muscles with associated areflexia. 1,2,6 Although our patient never developed bulbar palsy during his hospital course (rather, weakness in his diaphragm that required intubation and ventilation), the fact that he had initial upper extremity weakness raised the question as to whether he was suffering from PCB syndrome or perhaps with a classical GBS crossover. Our patient tested negative for anti-GT1a IgG antibodies, 1 which are the antiganglioside associated with the PCB variant, making an atypical presentation of AMAN more likely, although at this point it is impossible to say with certainty whether there were any PCB variant involvement.…”

“…Instead, patients present with a triad of ophthalmoplegia, ataxia, and areflexia. 1,2,6 As our patient did have severe limb involvement and did not have ophthalmoplegia, MFS (or a MFS/classical GBS crossover) was not highly suspected in our patient. Furthermore, our patient did not test positive for the antiganglioside antibody most often associated with the MFS variant, namely the anti-GQ1b antibody 1,2 -found positive in 80%-85% of MFS patients.…”

“…Rapidly progressive bilateral weakness is the main symptom a GBS patient will present with, typically ascending from the lower limbs to the upper extremities, [1][2][3][4][5][6][7] although others may present with cranial nerve involvement, or with bulbar, or autonomic dysfunction at first. For diagnosis of GBS, progressive weakness and areflexia must be present.…”

“…The demyelinating, referred to as acute inflammatory demyelinating polyneuropathy (AIDP), makes up 85%-90% of all classical GBS cases in the Western Hemisphere and presents with symmetric lower limb involvement with rostral progression, ataxia, and areflexia. 2,6 The axonal subtype or acute motor axonal neuropathy (AMAN) makes up roughly 5% of GBS cases and presents in a similar pattern as AIDP albeit with a faster progression and greater severity of motor weakness, ataxia, and areflexia. 2,8 The severity of the AMAN subtype may have to do with its associated antiganglioside antibodies (specifically the anti-Ga1NAc-GD1a/anti-GD1a) which target periaxonal membranes of motor nerves at the nodes and paranodes.…”

Epidemiologia da Paralisia Flácida Aguda no Brasil de 2014 a 2018

“…A Paralisia Flácida Aguda (PFA) é uma síndrome clínica caracterizada por arreflexia, hipotonia, alterações de despolarização que geram espasmos musculares e que podem cursar com atrofia muscular (BRASIL, 2013). As principais causas de PFA desde a erradicação da Poliomielite no Brasil incluem a Síndrome de Guillain-Barré (SGB), mielite transversa aguda (MTA), encefalomielite disseminada aguda, mielite infecciosa, botulismo, miastenia grave, traumatismos, distúrbios metabólicos e degenerativos, sendo a SGB e a MTA as etiologias mais frequentes (VASCONCELOS, 2017).…”