Volume 19, Number 9—September 2013
Synopsis
Detection of Diphtheritic Polyneuropathy by Acute Flaccid Paralysis Surveillance, India
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.
1Current affiliation: Massachusetts General Hospital, Boston, Massachusetts, USA.