Acute motor sensory axonal neuropathy complicated by multiple

Acute motor sensory axonal neuropathy complicated by multiple

PsychogeriatrIA PolSKA 2015;11(4):119-124
opis przypadku case report
Acute motor sensory axonal neuropathy
complicated by multiple organ dysfunction
syndrome: a case report
Ostra ruchowo-czuciowa neuropatia zapalna powikłana
zespołem niewydolności wielonarządowej: opis przypadku
Tomasz M. Gondek1, Anna Skoczyńska2, Ewa Gluza2,
Anna Zacharzewska-Gondek3
1
Department of Psychiatry, Wroclaw Medical University, Wroclaw, Poland
2Clinic of Internal and Occupational Diseases and Hypertension, University Hospital, Wroclaw, Poland
3
Department of General Radiology, Interventional Radiology and Neuroradiology, Wroclaw Medical University, Wroclaw, Poland
Key words: acute motor sensory axonal neuropathy (AMSAN); axonal polyneuropathy; multiple organ dysfunction syndrome (MODS); multiple organ failure (MOF); lower extremities paralysis; lower limbs paralysis; nerve conduction study (NCS); intravenous immuno
globulin (IVIg)
Słowa kluczowe:ostra ruchowo-czuciowa neuropatia zapalna, polineuropatia aksonalna, zespół niewydolności wielonarządowej, niewydolność wielonarządowa, porażenie kończyn dolnych, elektroneurografia (ENG), immunoglobuliny dożylne (IVIg)
PGP 194
Abstract
We describe the case of a 69-year-old woman with a history of hypertension and hypothyreosis, who
suffered from acute motor sensory axonal neuropathy (AMSAN), during which an immune-mediated
multiple organ dysfunction syndrome (MODS) developed. On admission the patient presented fever,
headache, adynamia, muscle weakness, pulmonary haemostasis and oedema of lower extremities. Bilateral pneumonia was diagnosed and confirmed radiologically. Laboratory tests showed: D-dimer, creatine
kinase and liver enzymes levels elevated; as well as hypokalaemia, hypocalcaemia, hypoproteinaemia
with hypoalbuminaemia and microhaematuria. Thorax CT angiography ruled out pulmonary embolism.
Echocardiography, abdominal ultrasonography, lower-extremity Doppler and Doppler of the supra-aortic
vessels were negative. Lower limbs paralysis led to an inflammatory polyneuropahty suspicion. Nerve
conduction study (NCS) revealed an axonal polyneuropahty with involvement of the peripheral nerves’
root segments, active process of muscle denervation but no features of a primary muscle damage.
Clinical picture combined with NCS results indicated AMSAN. Intravenous immunoglobulin (IVIg) was
administered and resulted in rapid, significant improvement of patient’s general condition and motor
function, while further improvement was achieved with rehabilitation.
Tomasz M. Gondek
Department of Psychiatry, Wroclaw Medical University
Wyb. Pasteura 10, 50-367 Wrocław, POLAND
Copyright © 2012 Fundacja Ochrony Zdrowia Psychicznego
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Tomasz M. Gondek Acute motor sensory axonal neuropathy complicated by multiple organ dysfunction
Streszczenie
Opisujemy przypadek 69-letniej kobiety, z rozpoznanym uprzednio nadciśnieniem tętniczym i niedoczynnością tarczycy, u której wystąpiła ostra ruchowo-czuciowa neuropatia zapalna (ang. acute motor
sensory axonal neuropathy, AMSAN), w przebiegu której rozwinęła się niewydolność wielonarządowa.
Przy przyjęciu do szpitala pacjentka miała gorączkę, skarżyła się na ból głowy, osłabienie i utratę siły
mięśniowej, obecne były cechy zastoju płucnego i obrzęki kończyn dolnych. Rozpoznano i potwierdzono
radiologicznie obustronne zapalenie płuc. Badania laboratoryjne wskazywały podwyższone poziomy:
D-dimerów, kinazy kreatynowej i enzymów wątrobowych; a także hipokaliemię, hipokalcemię, hipoproteinemię z hipoalbuminemią oraz krwinkomocz. Na podstawie angio-TK klatki piersiowej wykluczono
zatorowość płucną. Echokardiografia, ultrasonografia jamy brzusznej oraz badania dopplerowskie żył
kończyn dolnych oraz tętnic dogłowowych nie wykazały zmian chorobowych. Ze uwagi na rozwinięcie
się porażenia kończyn dolnych wysunięto podejrzenie polineuropatii zapalnej. Elektroneurografia (ENG)
wykazała obraz polineuropatii aksonalnej z zajęciem odcinków kostnych korzeniowych nerwów obwodowych i czynnym procesem odnerwienia mięśni, jednak bez pierwotnego uszkodzenia mięśni. Obraz
kliniczny w połączeniu z wynikiem ENG wskazywał na AMSAN. Do leczenia włączono immunoglobuliny
dożylne (IVIg), co poskutkowało szybką i znaczącą poprawą stanu ogólnego i funkcjonowania układu
ruchu, zaś dalszą poprawę uzyskano poprzez rehabilitację.
Introduction
Motion and touch, two natural features of a human being, have been a subject of philosophical
deliberation since its early days. According to Aristotle, motion, also in its most common meaning
of a physical move, was the transition from potentiality to actuality. In polyneuropathies, the potentiality,
i.e. the ability to move, is being gradually lost over time, which in some cases coexists with sensory
loss. For the ancients the loss of potentiality was irreversible, today however, in cases of certain acute
polyneuropathies, we are able to restore it.
Acute motor sensory axonal neuropathy (AMSAN) is a primary axonal subtype of Guillain-Barré syndrome (GBS), one of various forms of a wide spectrum of aquired axonal polyneuropathies.[1] While
its origin is still unknown, it is believed to be associated with certain viral and bacterial infections,
vaccination or surgical procedures, particularly spinal interventions.[2,3,4] Those incidents in a susceptible person can trigger both humoral and cell-mediated autoimmunological response against neuronal
components in a molecular mimicry mechanism, due to the similarity between them and the antigens
of microorganisms.[5] Recent findings indicate the main pathophysiological factor, common for the
whole spectrum of neuropathies associated with antibodies to the following antigens: GM1, GD1a and
GD1b; is a complement-mediated damage to nodes of Ranvier to varying degree.[6] AMSAN, along
with a more common acute motor axonal neuropathy (AMAN), is characterised by a rapid progression of neurological symptoms and a more severe course of the disease as well as a slower reaction
to therapy in comparison with acute inflammatory demyelinating polyradiculoneuropathy (AIDP), the
classic type of GBS.[7] The onset of the disease usually occurs 2 to 4 weeks after an upper respiratry
tract or gastrointestinal tract infection or a medical procedure, presenting paresthesia within the lower
extremity, followed by its paresis and the decrease of reflexes. It may lead to an ascending paralysis,
particularly dangerous if the respiratory muscles are affected, which usually results in a respiratory
failure.[7] The treatment of choice is either administration of intravenous immunoglobulin or plasmapheresis, both similarly succesful.[8]
A woman who we describe has recovered from a severe, life-threatening case of AMSAN, complicated
by an immune-mediated multiple organ dysfunction syndrome and other chronic disorders, with intravenous immunoglobulin administration.
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Tomasz M. Gondek Acute motor sensory axonal neuropathy complicated by multiple organ dysfunction
Case report
A 69-year-old woman with a long history of hypertension and a post-operative hypothyreosis has been
admitted to our clinic via an emergency route because of a persistent fever of 39°C that have lasted for
about 3 weeks and intensifying weakness accompanied by a severe headache. Headaches have been
occurring for several weeks which prompted the patient to look for a diagnosis. In order to clarify their
cause, the patient had previously been hospitalized in the neurology department, where an aneurysm,
vascular malformation or CNS inflammatory process had been ruled out, but tension headache had
been diagnosed and treated with Opipramol and Diclofenac, further observation by GP and a psychiatric
consultation in mental health centre had also been recommended.
On admission to our Clinic the patient was in a severe general condition with adynamia, muscle
weakness, pulmonary haemostasis and massive oedema of lower extremities. The anamnesis indicated
that, prior to the hospitalization, the patient has not been taking statins or fibrates but only has been
taking: Euthyrox, Acard and Tertensif SR. The patient was diagnosed with bilateral pneumonia, which
was confirmed radiologically. Because of the coincidence of dyspnea and very high D-dimer values,
pulmonary embolism has been suspected but has ultimately been ruled out by thorax computed tomography angiography. Tests for atypical pneumonia were negative. Transthoracic echocardiography
was performed but showed no bacterial vegetations. Laboratory blood test results, apart from high
D-dimer, showed creatine kinase and liver enzymes levels elevated, as well as hypokalaemia, hypocalcaemia and low levels of protein and albumin. Hypokalaemia was treated with intravenous and oral
potassium supplementation and with administration of spironolactone. Urinalysis showed microscopic
haematuria. Abdominal ultrasonography revealed no abnormalities, neither did lower-extremity Doppler,
while Doppler of the supra-aortic vessels only found that intima-media complex in the carotid bulbs
was thickened to 1 mm.
On the fourth day of hospitalization a neurological consultation was performed and it was ascertained
the patient was conscious, maintained logical verbal contact, there were no meningeal signs and
symptoms, but a fatigable nystagmus when looking at both sides was observed. The strength of the
upper limbs was good but reflexes were poor and there was a paralysis of the lower limbs – the patient
couldn’t detach legs from the ground, there were no deep tendon reflexes but no sensory disturbances
were present, except for quadriceps muscles being painful on palpation. On the basis of anamnesis
and physical examination, an inflammatory polyneuropathy was suspected, which was confirmed with
nerve conduction study (NCS).
The NCS has shown: (I) a prolonged F-wave latency in the examined nerves, in some nerves
the F-response has not been obtained; (II) low amplitudes of motor responses in all examined nerves,
particularly in the lower extremities with a clear tendency for further amplitude reduction in the proximal segments, normal motor conduction velocity and prolonged end latency in the left median nerve
with a considerably decreased motor conduction velocity at the wrist; (III) low amplitudes of sensory
responses in the majority of examined nerves, decreased sensory conduction velocity in the median
nerve at wrist. The NCS has also shown an increased rest activity in the left tibialis anterior muscle
and increased polyphasic potentials, while other parameters of motor units in both examined muscles
were within normal limits; there were no typical neurogenic changes. The examination have indicated an axonal polyneuropathy with marked involvement of the peripheral nerves’ root segments and
an active process of muscle denervation. There have been no apparent features of a primary muscle
damage. This examination had led to a strong suspiction of AMSAN and, combined with clinical picture,
allowed to set the diagnosis.
Intravenous immunoglobulin (IVIg) in dose of 0,4 g/kg BW for 5 consecutive days has been administered.
During the course of treatment, the patient received plasma and 20% albumin solution, protein was
also supplemented enterally with high-protein preparations. Euthyrox supplementation was modified,
combined diuretic therapy with blood pressure control was applied and fluid levels were maintained
throughout the therapy. The treatment brought rapid and significant improvement of patient’s clinical
condition, confirmed also in the second neurological consultation and resulted in normalization of the
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Tomasz M. Gondek Acute motor sensory axonal neuropathy complicated by multiple organ dysfunction
Fig. 1. Laboratory tests results during the course of hospitalization (I)
Ryc. 1 Wyniki badań laboratoryjnych w przebiegu hospitalizacji (I)
Fig. 1. Laboratory tests results during the course of hospitalization (II)
Ryc. 1 Wyniki badań laboratoryjnych w przebiegu hospitalizacji (II)
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Tomasz M. Gondek Acute motor sensory axonal neuropathy complicated by multiple organ dysfunction
majority of laboratory parameters. The patient was rehabilitated in our Clinic during the hospitalization
and continuation of rehabilitation after the discharge was recommended in order to further improve
motor function. Remaining under constant care of GP and neurological clinic was also recommended.
7 months later the patient has been hospitalized in our Clinic because of chirarthritis and tachycardia
and her general condition, as well as motor function, was significantly better than at the time of the
previous discharge from the hospital.
Discussion
It is suggested AMSAN is not an autonomous disease, but is rather a variant within a spectrum
of autoimmunological neuropathies of common pathophysiology, along with AMAN and multifocal motor
neuropathy (MMN), which are separated not only by the neurological symptoms they cause, but also
by the severity of autoimmunological response.[9] Considering the fact that motor neurons are more
vulnerable to the antibody response in comparison to sensory neurons, in milder cases only motor
symptoms may be present, while in AMSAN motor symptoms may predominate over sensory deficits;
it may also explain why AMAN is considerably more frequent than AMSAN.[5,9] Observations of our
patient, who clinically did not present any sensory deficit but low amplitudes of sensory responses were
only revealed in the nerve condution study, whereas significant motor disorder and general severe
condition were diagnosed, are consistent with those reports.
Electrophysiologic findings in our patient are typical for AMSAN, however AIDP may show similar aberrations when performed in early course of the disease.[7] Nevertheless, NCS was crucial in setting the
diagnosis, as the clinical picture of rapidly developing symptoms allowed to rule out the most common
form of GBS, which tends to progress significantly slower, over a period of few weeks in comparison
to days in AMSAN.[7] Following serial nerve condution studies would be necessary in order to electrophysiologically confirm the diagnosis.[10]
The multi-organ dysfunction, which have developed during the course of the illness and led to extremely
severe condition, withdrew almost completely with intravenous immunoglobulin administration in dose
of 0,4 g/kg BW for 5 consecutive days. Such a rapid recovery is uncommon for AMSAN , as usually
the recovery takes a longer period in comparison to AIDP and may be incomplete.[11] There was also
a significant recovery of motor functions with a prognosis of further normalization after physical rehabilitation. None of the frequent side effects of IVIg,[12] other than headaches, which the patient has
already suffered from previously (and their frequency or severity did not change during the therapy),
were observed in our patient during or after the therapy. Alternatively to this course of treatment,
it is reported plasmapheresis can be performed to similar effect, while glucocorticoids, despite their
immunosuppressive action in high doses, are not recommended.[8] However, there are case reports
in which authors present a succesful recovery from AMSAN on high-dose glucocorticoids combined with
IVIg and low-dose intravenous cyclophosphamide pulses.[13]
We believe that the case we report is valuable as it shows how rapid and how considerable the improvement of the patient’s state can be after an accurate therapy is administered in this disorder.
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Tomasz M. Gondek Acute motor sensory axonal neuropathy complicated by multiple organ dysfunction
Piśmiennictwo
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[2] Hughes RA, Hadden RD, Gregson NA, Smith KJ Pathogenesis of Guillain-Barré syndrome. J. Neuroimmunol. 1999 Dec;100(1-2):74-97
[3] Vital C, Vital A, Gbikpi-Benissan G, Longy-Boursier M, Climas MT, Castaing Y et al. Postvaccinal
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[4] Miscusi M, Currà A, Della Rocca C, Missori P, Petrozza V Acute motor-sensory axonal neuropathy
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[9] Uncini A, Notturno F, Capasso M Natura non facit saltus in anti-ganglioside antibody-mediated
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[10]Uncini A, Yuki N Electrophysiologic and immunopathologic correlates in Guillain-Barré syndrome
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[11]Akbayram S, Doğan M, Akgün C, Peker E, Sayιn R, Aktar F et al. Clinical features and prognosis
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[12]Duhem C, Dicato MA, Ries F Side-effects of intravenous immune globulins. Clin. Exp. Immunol. 1994
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[13]Santiago-Casas Y, Peredo RA, Vilá LM Efficacy of low-dose intravenous cyclophosphamide in systemic lupus erythematosus presenting with Guillain-Barre syndrome-like acute axonal neuropathies:
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Revieved/Zrecenzowano 21.07.2015r.
Accepted/Zatwierdzono do druku 21.07.2015r.