# Acute Inflammatory Demyelinating Polyneuropathy and a Unilateral

Authors: Davide Cattano; Brian O'connor; Ra'ad Shakir; Francesco Giunta; Mark Palazzo
Journal: Anesthesiology Research and Practice
 (2008)

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## Abstract

Acquired acute demyelinating peripheral
polyneuropathy (AADP) is a general classification of pathologies
that could affect secondary the peripheral nervous system. They
are characterized by an autoimmune process directed towards
myelin. Clinically they are characterized by progressive weakness
and mild sensory changes. Acute inflammatory demyelinating
polyneuropathy often is referred to as Guillain-Barré syndrome
(GBS). GBS is the major cause of acute nontraumatic paralysis in
healthy people and it is caused by autoimmune response to viral
agents (influenza, coxsackie, Epstein-Barr virus, or
cytomegalovirus) or bacterial infective organisms (Campylobacter
jejuni, Mycoplasma pneumoniae). A detailed history, with symptoms
of progressive usually bilateral weakness, hyporeflexia, with a
typical demyelinating EMG pattern supports the diagnosis.
Progressive affection of respiratory muscles and autonomic
instability coupled with a protracted and unpredictable recovery
normally results in the need for ICU management. We present a case
report of a patient with a typical GBS presentation but with a
unilateral upgoing plantar reflex (Babinski sign). A unifying
diagnosis was made and based on a literature search in Pubmed
appears to be the first described case of its kind.


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## Body

## 1. Discussion

This diabetic patient initially presented with a history of general weakness and a fever
which on initial examination and results of investigations fitted in with a
diagnosis ofS. aureus septic shock of uncertain origin complicated by EMG-proven AIDP. Unusually, the patient also repeatedly had a clinically elicited unilateral
Babinski response with no other upper motor neuron signs or central disturbance.
In view of the previous history of back pain and absent central symptoms, this was thought to be
possibly related to a coincidental cervical lesion. However, initial spinal
radiology was normal.However, it was the sudden development of dysarthria which led to a unifying diagnosis of acute
bacterial endocarditis unusually complicated by acute inflammatory demyelinating polyneuropathy and cerebral emboli. This produced clinical signs of upper and lower motor neuron lesions.The association of bacterial endocarditis with both AIDP and cerebral embolic infarcts has not
been previously described. Acute bacterial endocarditis is complicated by
neurological changes in 25–40% of cases [1–4]. The most common neurological complications are central causing upper motor neuron changes and they are most frequently
represented by cerebral embolism, meningitis, micrabscess, and mycotic aneurysm with or without subsequent haemorrhage. Neurological complications seem to be more frequent when S. aureus
is the infecting organism [1]. Global peripheral nervous involvement is much less common and if peripheral nervous changes are observed, it is usually an acute mononeuropathy [4].
There have been however reports of axonal polyneuropathy or critical illness
polyneuropathy associated with acute bacterial endocarditis, but these are
usually a specific manifestation of established multiple organ failure [5–7].Our patient presented with clinical and EMG findings typical of AIDP, which can be
associated with any number of infections, but a rare manifestation of acute
bacterial endocarditis. It was sufficiently severe to result in progressive type
2 respiratory failure, but it responded well to a course of immunoglobulin such
that he was weaned from ventilation in a few days.The unilateral Babinski response was difficult to account for in the absence of evidence of
central or high spinal lesions. AIDP typically affects peripheral and cranial
nerves but not the CNS [8] and therefore it is unlikely to be the cause of our patient's unilateral Babinski response.This report illustrates that when an unusual constellation of clinical signs is observed, a
unifying diagnosis should always be sought. In this case, the rarer global
acute demyelinating lower motor neuron changes dominated, mostly disguised the
upper motor neuron signs of hyperreflexia, and increased tone usually
associated with a Babinski response.The second lesson from this case is that history taking is usually better once a diagnosis is
made.Suspicion would have been heightened and cardiac investigations were performed earlier with
a directed history for a patient with pyrexia of unknown origin. Fortunately,
our patient received early mitral valve replacement and was discharged home.



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*Source: 134958-2007-11-07.xml*