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Volume 19, Number 9—September 2013

Synopsis

Detection of Diphtheritic Polyneuropathy by Acute Flaccid Paralysis Surveillance, India

Farrah J. Mateen1Comments to Author , Sunil Bahl, Ajay Khera, and Roland W. Sutter
Author affiliations: Johns Hopkins University, Baltimore, Maryland, USA (F.J. Mateen); World Health Organization, Geneva, Switzerland (F.J. Mateen, R.W. Sutter); World Health Organization National Polio Surveillance Project, New Delhi, India (S. Bahl); Ministry of Health and Family Welfare, New Delhi (A. Khera)

Main Article

Table 1

Clinical characteristics of 15 children with diphtheritic polyneuropathy, India, 2007–2011*

Patient Age, y/sex Paralysis description (worst motor power) Tones/reflexes Fever at onset Neck swelling Other symptoms of diphtheria CSF/NCS Respiratory involvement GBS disability score Outcome at 60-d follow-up (no. days death occurred postparalysis onset)
1 5/F Symmetric limb weakness (NA) NA N N Throat pain, nasal regurgitation, nasal intonation, and twang with speech NA Y 6 Initially improved to independent sitting, standing, and holding head; then died from presumed respiratory failure (46)
2 3/M Hypotonic, areflexic, symmetric weakness of all limbs (MRC 2) ↓/↓ Y N Neck flop, prior sore throat, nasal regurgitation, inability to speak NA Y 5 Ventilator dependent
3 4/M Descending, asymmetric lower extremity paralysis (MRC 4) ↓/NL N Y Nasal voice, nasal regurgitation of food, progressive weakness, and inability to walk after throat symptoms improved NA Y 6 Died from respiratory failure (15)
4 3/M Symmetric, hypotonic, areflexic paralysis of the extremities (MRC 3) ↓/↓ Y Y Nasal voice and regurgitation with feeding ≈15–20 d after neck swelling NA N 3 No clinical improvement, remained hyporeflexic and hypotonic in limbs
5 4/M Symmetric, hypotonic, diffuse weakness, unconscious (unable to test) ↓/↓ Y Y Hyponasal speech, difficulty swallowing, nasal regurgitation 20 d after neck swelling NA Y 6 Died from cardiorespiratory failure while ventilator dependent (36)
6 6/M Symmetric, hypotonic weakness, lower extremity weakness (MRC 3) ↓/↓ Y Y Neck swelling and fever for 13 d, flaccid paralysis developed 30 d later with persistent voice change NL/NA Y 6 Died from cardiorespiratory failure while ventilator dependent (18)
7 6/F Symmetric, descending, hypotonic, lower extremity weakness (MRC 4) ↓/NL Y Y Nasal regurgitation and speech twang with enlarged glands and cervical adenopathy; weakness 13 d later NL/NL Y 6 Died from unclear reasons, presumed cardiorespiratory failure (37)
8 6/F Symmetric, ascending, hypotonic weakness (MRC 3) ↓/↓ Y Y Nasal twang, swallowing difficulty for 1 mo, then weakness involving legs and hands for <1 wk NA N 3 Strength improved by 1 point on MRC scale in upper and lower extremities
9 6/M No limb weakness (MRC 5) NL/NL Y Y Fever for 2 d, then sudden voice change with nasal regurgitation for 2–3 d, before pain and swelling of neck 2 weeks earlier NA N 0 Persistent palatal palsy
10 2/F Symmetric, descending, hypotonic weakness (MRC 4) ↓/↓ Y N Fever followed by nasal regurgitation and difficulty swallowing, progressive weakness in all limbs developed 2 d later NA N 4 Able to stand but requires support to walk
11 4/M Symmetric, hypotonic, lower worse than upper extremity weakness with prominent sensory symptoms (MRC 3 and 4) ↓/↓ N Y Throat and bulbar symptoms preceding limb weakness; throat swab negative NL/demyelinating Y 6 Serum given without noticeable improvement; died from cardiorespiratory arrest (13)
12 4/M Descending, symmetric, lower extremity predominant weakness (MRC 4) ↓/↓ N Y Nasal regurgitation preceding weakness NA/mixed axonal and demyelinating Y 6 Died from respiratory failure (21)
13 5/F Symmetric, diffuse, hypotonic weakness (MRC 4) ↓/↓ Y Y Nasal regurgitation NL/NA Y 6 Died from presumed cardiorespiratory failure (6)
14 14/M Complete flaccidity, areflexia, atonia; EMG showed no spontaneous motor activity or recruitment of motor unit potentials (MRC 0) ↓/↓ Y N History of positive throat swab result for Corynebacterium diphtheria NL/demyelinating Y 5 No spontaneous muscle activity; no response to IVIg
15 2/F Symmetric, 4-limb, lower extremity, predominant weakness (MRC 3 in lower extremities) NL/↓ N N Nasal regurgitation, speech change, and difficulty swallowing with fever, paralysis in <7 d NL/demyelinating N 4 Unable to walk

*CSF, cerebrospinal fluid; NCS, nerve conduction study; GBS, Guillain-Barré syndrome; NA, not available; N, no; Y, yes; MRC, Medical Research Council scale score; ↓, decreased or absent deep tendon reflexes; NL, normal; EMG, electromyogram; IVIg, intravenous immunoglobulin.

Main Article

1Current affiliation: Massachusetts General Hospital, Boston, Massachusetts, USA.

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