ArticlePDF Available

Diphtheritic polyneuropathy in the wake of resurgence of diphtheria

October 2015
Journal of Pediatric Neurosciences 10(4):331

October 2015
10(4):331

License
CC BY-NC-SA 3.0

Authors:

Objective: To study the clinical profile and outcome in children with diphtheritic polyneuropathy (DP). Methodology: 13 children with polyneuropathy were included in this study. Their demographic profile, age, sex and immunization status were recorded. Detailed clinical and neurological examination was done. Investigations like CSF analysis, NCV studies, MRI brain were done. The results were tabulated and analyzed. Results: All the children presented with bulbar palsy and had h/o membranous tonsillitis. Isolated palatal palsy was seen in 7 children (53%). 6 (46.1%) children developed quadriparesis. 1 child expired and recovery is complete in rest of the 12 children. Children with isolated bulbar palsy recovered within 2 to 4 weeks while children with quadriparesis recovered within 5-6 wks. Conclusions: Any child diagnosed with diphtheria should be followed for 3-6 months in anticipation of neurological complications. DP carries good prognosis hence timely diagnosis and differentiation from other neuropathies is a prerequisite for rational management.

Available via license: CC BY-NC-SA 3.0

Content may be subject to copyright.

Original Article

Diphtheritic polyneuropathy in the wake of

resurgence of diphtheria

D. Manikyamba, A. Satyavani, P. Deepa

Department of Pediatrics, Rangaraya Medical College, Kakinada, Andhra Pradesh, India

Address for correspondence: Dr. D. Manikyamba, Department of Pediatrics, Rangaraya Medical College, Kakinada, Andhra Pradesh, India.

E‑mail: manirmurthy@gmail.com

ABSTRACT

Objective: To study the clinical profile and outcome in children with diphtheritic polyneuropathy (DP).

Methodology: 13 children with polyneuropathy were included in this study. Their demographic profile, age, sex

and immunization status were recorded. Detailed clinical and neurological examination was done. Investigations

like CSF analysis, NCV studies, MRI brain were done. The results were tabulated and analyzed. Results: All

the children presented with bulbar palsy and had h/o membranous tonsillitis. Isolated palatal palsy was seen

in 7 children (53%). 6 (46.1%) children developed quadriparesis. 1 child expired and recovery is complete in

rest of the 12 children. Children with isolated bulbar palsy recovered within 2 to 4 weeks while children with

quadriparesis recovered within 5‑6 wks. Conclusions: Any child diagnosed with diphtheria should be followed

for 3‑6 months in anticipation of neurological complications. DP carries good prognosis hence timely diagnosis

and differentiation from other neuropathies is a prerequisite for rational management.

Key words: Anti‑diphtheritic serum, complications, diphtheria, polyneuropathy

Introduction

Diphtheritic polyneuropathy (DP) is recognized as one of the

most severe complications of diphtheria, caused by exotoxin of

Corynebacterium diphtheriae. Among 20,000 cases of diphtheria

reported by WHO during 2007–2011, 17,926 (89.6%) cases

were from India alone.[1] Most persons with diphtheria in India

are either partially immunized or unimmunized.

Scarce reports of DP in the Indian literature may result from

under-recognition and perhaps, under-reporting of this entity.

In this paper, we present a case series of 13 children with DP

from South India admitted in our hospital from July 2013 to

December 2013.

During the same period, 19 children admitted with membranous

tonsillitis in our hospital were identified as probable or

confirmed cases of diphtheria with culture positivity in

13 children. They were treated with anti-diphtheritic

serum (ADS) and antibiotics. Among 19 children, 7 children

expired and the rest were followed for 6 months.

The simultaneous resurgence of clinical diphtheria in our area

helped us probe into the history of those children who presented

with neuropathy and throw light on this latent entity which is

almost forgotten by the present day physicians and neurologists.

In India, apart from the case series from Delhi, there is no other

case series reported so far in the recent past.

Methods

Thirteen children with DP were included in this study. Their

Cite this article as: Manikyamba D, Satyavani A, Deepa P. Diphtheritic

polyneuropathy in the wake of resurgence of diphtheria. J Pediatr Neurosci

2015;10:331-4.

This is an open access article distributed under the terms of the Creative

Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows

others to remix, tweak, and build upon the work non-commercially, as long as the

author is credited and the new creations are licensed under the identical terms.

For reprints contact: reprints@medknow.com

Access this article online

Quick Response Code:

Website:

www.pediatricneurosciences.com

DOI:

10.4103/1817-1745.174441

[Downloaded free from http://www.pediatricneurosciences.com on Wednesday, September 28, 2016, IP: 91.248.105.162]

Manikyamba, et al.: Diphtheritic polyneuropathy

332 / Journal of Pediatric Neurosciences / Volume 10 / Oct-Dec / 2015

demographic profile, age, sex, and immunization status were

recorded. Detailed clinical and neurological examination

was done. Investigations such as cerebrospinal fluid (CSF)

analysis, nerve conduction velocity (NCV) studies, Magnetic

resonance imaging (MRI) brain were carried out. The

results were tabulated and analyzed. Among 13 children,

10 presented with neuropathy whose past histories revealed

membranous tonsillitis. Three children presented to us with

respiratory diphtheria and developed neuropathy during

follow-up.

Results

Children in the present series were in the age group of

5–13 years. All the children had history of membranous

tonsillitis with a latency period of 15–40 days between the

onset of tonsillitis and neurological symptoms.

All 13 children had bulbar palsy. Isolated palatal palsy was

seen in 7 children (53%). Oculomotor nerve involvement in

the form of accommodation paralysis was seen in 3 children.

One child had unilateral lower motor neuron facial palsy and

6 children developed quadriparesis after palatal palsy, which

was descending and symmetric in nature [Table 1].

Supportive treatment with Ryle’s tube feeding was given to

all children for 1–4 weeks.

One child expired during hospital stay due to aspiration.

Recovery was complete in rest of the 12 children.

Children with isolated bulbar palsy recovered within 2–4 weeks

while children with quadriparesis recovered within 5–6 weeks.

Discussion

The incidence of neurologic complications is related directly

to the severity of respiratory symptoms. DP is seen in 20% and

75% of patients with mild and severe infection, respectively.[2]

Table 1: Details of children with DP

Age/sex Immunization

status*

Central nervous system ndings Course of the disease after

membranous tonsillitis

Investigations: CSF‡, NCV§, MRI||

13/female Unimmunized Hypotonia,areexia,power‑3/5

Cranial nerve palsies: 3rd, 7th, 9th, 10th

3rd week-bulbar palsy

4th week-quadriparesis

16th week-recovery of speech

20th week-recovery of weakness

CSF: Protein elevation

NCV: Axonal degeneration

MRI: Normal

11/female Unimmunized Hypotonia,areexia,power‑3/5

Cranial nerve palsies: 9th, 10th

4th week-nephrosis, myocarditis

5th week-bulbar palsy

6th week-quadriparesis

8th week-recovery of speech

12th week-recovery of weakness

CSF: Protein elevation

NCV: Axonal degeneration

MRI: Normal

9/female†Partially

immunized

Hypotonia,areexia,power‑3/5

Cranial nerve palsies: 3rd, 6th, 9th,

10th

3rd week-bulbar palsy, blurred

vision, diplopia

4th week-quadriparesis

6th week-expired

CSF: Protein elevation

NCV: Axonal degeneration

MRI: Normal

8/female Unimmunized Hypotonia,areexia,power‑3/5

Cranial nerve palsies: 9th, 10th

5th week-bulbar palsy

7th week-quadriparesis

10th week-recovery of speech

12th week-recovery of weakness

CSF: Protein elevation

NCV: Axonal degeneration

MRI: Normal

9/female Unimmunized Hypotonia,areexia,power‑3/5

Cranial nerve palsies: 9th, 10th

2nd week-bulbar palsy

4th week-recovery

CSF: Protein elevation

NCV: Demyelinating variety

MRI: Normal

11/female Partially

immunized

Hypotonia,reexes+,power‑4/5

Cranial nerve palsies: 9th, 10th

4th week-bulbar palsy

7th week-recovery

Not done

9/male Partially

immunized

Normaltone,reexes+,power‑5/5

cranial nerve palsies: 9th, 10th

3rd week-bulbar palsy

7th week-recovery

Not done

12/female Unimmunized Normaltone,reexes+,power‑5/5

Cranial nerve palsies: 9th, 10th

4th week-bulbar palsy

7th week-recovery

Not done

5/female Partially

immunized

Normaltone,reexes+,power‑5/5

Cranial nerve palsies: 9th, 10th

5th week-bulbar palsy

10th week-recovery

Not done

11/male Partially

immunized

Normaltone,areexia,power‑5/5

Cranial nerve palsies: 9th, 10th

6th week-bulbar palsy

10th week-recovery

CSF: Protein elevation not seen

NCV and MRI not done

10/male Unimmunized Normaltone,areexia,power‑5/5

Cranial nerve palsies: 9th, 10th

5th week-bulbar palsy

8th week-recovery

Not done

9/female Partially

immunized

Normaltone,areexia,power‑5/5

Cranial nerve palsies: 9th, 10th

7th week-bulbar palsy

10th week-recovery

Not done

8/male Unimmunized Normaltone,areexia,power‑5/5

Cranial nerve palsies: 9th, 10th

6th week-bulbar palsy

10th week-recovery

Not done

*6 (47%) children were partially immunized and 7 (53%) children were unimmunized, †C diphtheriae was isolated from throat swab culture in this child, ‡CSF analysis revealed

albumino-cytological dissociation in 4 patients, §NCV showed axonal degeneration in 4 children and demyelination in 2 children with quadriparesis, ||MRI brain and spine was done in

childrenwithquadriparesisanditwasnormalinallofthem.DP:Diphtheriticpolyneuropathy,MRI:Magneticresonanceimaging,NCV:Nerveconductionvelocity,CSF:Cerebrospinaluid

[Downloaded free from http://www.pediatricneurosciences.com on Wednesday, September 28, 2016, IP: 91.248.105.162]

Manikyamba, et al.: Diphtheritic polyneuropathy

2015 / Oct-Dec / Volume 10 / Journal of Pediatric Neurosciences / 333

The period between the appearance of first symptom of

diphtheria and the development of DP is termed latency,

which varies from 10 days to 3 months.[3] The first indication

of neuropathy is paralysis of the soft palate and posterior

pharyngeal wall.[4] Bulbar dysfunction typically develops

during the first 2 weeks. Oculomotor and ciliary paralyses

seen after 3 weeks are common and distinctive features of DP.

Peripheral neuritis develops later, from 10 days to 3 months

after the onset of oropharyngeal disease. In some patients,

there may be a secondary worsening of the bulbar symptoms

along with the occurrence of peripheral neuropathy.[2]

Biphasic course of disease was noted only in 2 children in our

study which is contradictory to other studies.

Various studies on major epidemic outbreaks from Russia and

Europe in the past gave us valuable insights on pathophysiology

and progression of DP. Diptheritic toxin penetrates into

Schwann cells and it inhibits the synthesis of myelin

proteolipid and basic protein.[4] It was observed in vitro that it

binds to Schwann cells as early as 1-hr postinjection, inducing

the latent development of polyneuropathy.[5] This stresses the

importance of immediate administration of ADS. Local toxic

effects occur by direct spread of toxin and result in the early

bulbar problems while the ensuing generalized demyelinating

neuropathy arises from hematogenous dissemination.[6] The

reason behind isolated palatal palsy in few children with no

systemic involvement is probably nondissemination of the

toxin.

DP has to be distinguished from other neuropathies especially

Guillain–Barre syndrome (GBS) which is more common in

children. The clinical features differentiating DP from GBS

are high prevalence of bulbar palsy, slower evolution of the

neuropathy for more than 4 weeks, descending nature, and

simultaneous involvement of other organ systems. NCV

studies and CSF findings are similar to those in GBS. The

management and prognosis of DP differs from GBS.[7]

Profile of DP in various studies is described in Table 2. In

2005, Logina and Donaghy reported an attenuated form

of neuropathy in the immunized population of Latvia. This

was attributed to the protective effects of the vaccine, which

attenuated the adverse effects of exotoxin. Compared to the

previous epidemic in 1999, the incidence and severity were

lower in same area due to early diagnosis and administration

of ADS within first 3 days of disease.[8] With this review of

literature and supportive evidence from our study, we stress

upon the recognition of epidemic in its incipient stage and

prompt administration of ADS.

Studies from Russia showed a significant trend of decreasing

immunity with increasing age, resulting in lack of protection,

particularly for adults aged 30 to 50 years. As vaccine

induced immunity wanes over time, periodic boosters are

recommended every 10 years. Thus the susceptibility of adults

to diphtheria is a new phenomenon of the vaccine era.[9]

In India, Mohanta and Parija reported 86 cases of DP in

children from Odisha in 1974.[10] Mild respiratory muscle

involvement was seen in one child in our study in contrast to

the study by Sandeep Kumar et al.(2010) from Delhi where

respiratory muscles were involved in 85.4% cases and 60.4%

required mechanical ventilation.[11] In 2013, Mateen et al.

reported a case series of 15 children with DP from nine

states and Union territories, detected through Acute flaccid

paralysis (AFP) surveillance [2002–2008]. Their study

demonstrates that DP can be detected through existing AFP

surveillance system.

Conclusions

Pediatricians/neurophysicians should have a high index of

suspicion to recognize DP in the wake of recent resurgence of

diphtheria in some parts of India. Any child diagnosed with

probable diphtheria should be followed for 3–6 months for

neurological complications.

As seen in our case series, DP carries good prognosis hence

timely diagnosis and differentiation from other neuropathies

is a prerequisite for rational management and contact

tracing.

Continued occurrence of diphtheria emphasizes the need for

public health measures such as:

Table 2: Features of DP in various studies

Our study AFP surveillance Delhi (2010) Latvia (1997) Latvia (2005)

Age group 5-13 <15 4.25 41-60 18-24

Immunization status

Complete 80%

Partial 47% 15%

Unimmunized 53% 100% 5%

Latency in days 15-30 10 10 43

Isolated palatal palsy 60% 15% 6% 10% 0

Limb involvement 40% 85% 94% 90% 100%

CSF analysis 80% protein elevation

20%-normal

100%-normal 100%-normal

Mechanical ventilation No 20% 60% No

Recovery (in days) 21-102 days 20-115 30

Fatal outcome 8% 46% 14.6% 16% 0

DP:Diphtheriticpolyneuropathy,AFP:Acuteaccidparalysis

[Downloaded free from http://www.pediatricneurosciences.com on Wednesday, September 28, 2016, IP: 91.248.105.162]

Manikyamba, et al.: Diphtheritic polyneuropathy

334 / Journal of Pediatric Neurosciences / Volume 10 / Oct-Dec / 2015

• Strengtheningofroutineimmunizationandmandatory

booster vaccination at school entry and Td booster[12] at

10 years intervals thereafter

• Vaccinationofsusceptiblecontactsandprophylactic

antibiotics to contacts

• EnsuringavailabilityofADSfortimelyadministration

and thereby preventing complications

• AFPsurveillancesystemcanbeutilizedtoidentifyDP

and thus areas of resurgence of diphtheria and strengthen

immunization services in those pockets.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1. Mateen FJ, Bahl S, Khera A, Sutter RW. Detection of diphtheritic

polyneuropathy by acute flaccid paralysis surveillance, India. Emerg

Infect Dis 2013;19:1368-73.

2. Hadfield TL, McEvoy P, Polotsky Y, Tzinserling VA, Yakovlev AA. The

pathology of diphtheria. J Infect Dis 2000;181 Suppl 1:S116-20.

3. Piradov MA, Pirogov VN, Popova LM, Avdunina IA. Diphtheritic

polyneuropathy: Clinical analysis of severe forms. Arch Neurol

2001;58:1438-42.

4. McAuley JH, Fearnley J, Laurence A, Ball JA. Diphtheritic

polyneuropathy. J Neurol Neurosurg Psychiatry 1999;67:825-6.

5. Pleasure DE, Feldmann B, Prockop DJ. Diphtheria toxin inhibits the

synthesis of myelin proteolipid and basic proteins by peripheral nerve

in vitro. J Neurochem 1973;20:81-90.

6. Solders G, Nennesmo I, Persson A. Diphtheritic neuropathy, an analysis

based on muscle and nerve biopsy and repeated neurophysiological

and autonomic function tests. J Neurol Neurosurg Psychiatry

1989;52:876-80.

7. Logina I, Donaghy M. Diphtheritic polyneuropathy: A clinical study

and comparison with Guillain-Barré syndrome. J Neurol Neurosurg

Psychiatry 1999;67:433-8.

8. Krumina A, Logina I, Donaghy M, Rozentale B, Kravale I, Griskevica A,

et al. Diphtheria with polyneuropathy in a closed community despite

receiving recent booster vaccination. J Neurol Neurosurg Psychiatry

2005;76:1555-7.

9. Hann AF. Resurgence of diphtheria in the newly independent states

of the former Soviet Union: A reminder of risk. J Neurol Neurosurg

Psychiatry 1999;67:426.

10. Mohanta KD, Parija AC. Neurological complications of diphtheria.

Indian J Pediatr 1974;41:237-43.

11. Kanwal SK, Yadav D, Chhapola V, Kumar V. Post-diphtheritic

neuropathy: A clinical study in paediatric intensive care unit of a

developing country. Trop Doct 2012;42:195-7.

12. World Health Organization. Diphtheria: Immunization Surveillance,

Assessment, and Monitoring. Available from: http://www.who.int/

immunization_monitoring/diseases/diphteria/en/index.html.[Last cited

on 2013 Mar 05].

Staying in touch with the journal

1) Table of Contents (TOC) email alert

Receive an email alert containing the TOC when a new complete issue of the journal is made available online. To register for TOC alerts go to

www.pediatricneurosciences.com/signup.asp.

2) RSS feeds

Really Simple Syndication (RSS) helps you to get alerts on new publication right on your desktop without going to the journal’s website.

You need a software (e.g. RSSReader, Feed Demon, FeedReader, My Yahoo!, NewsGator and NewzCrawler) to get advantage of this tool.

RSS feeds can also be read through FireFox or Microsoft Outlook 2007. Once any of these small (and mostly free) software is installed, add

www.pediatricneurosciences.com/rssfeed.asp as one of the feeds.

[Downloaded free from http://www.pediatricneurosciences.com on Wednesday, September 28, 2016, IP: 91.248.105.162]

Post-diphtheritic Polyneuropathy in Children with Diphtheria: A Prospective Observational Study

Article

Full-text available

Oct 2023

This study aimed to determine the proportion of diphtheria patients who develop post-diphtheritic polyneuropathy (DP) later on and also to study the clinical features and outcomes of children with post-DP. This prospective observational study was conducted at the Department of Pediatrics, King George’s Medical University, Lucknow, Uttar Pradesh, India. Children under 14 hospitalized with clinically diagnosed diphtheria and post-DP were recruited (n = 81). Detailed clinical examination with appropriate investigations was conducted, including throat swabs for staining and culture for Corynebacterium diphtheria, nerve conduction studies, electrocardiography, and echocardiography. The data were analyzed using Statistical Package for Social Sciences (SPSS) version 16.0. Seventy-four cases of diphtheria and seven cases of post-DP were enrolled, 56.8% were male, and the most prevalent age group afflicted was two to five years. Fifty-three children (65.4%) were partially immunized for diphtheria. Neck swelling, voice change, difficulty breathing, noisy breathing, respiratory involvement, and stridor were significantly more common in the unimmunized group. Voice change, heart rate irregularity, and hypotension were substantially more common in patients who developed clinical neuropathy than those who did not. Early administration of antibiotics in children with diphtheria before hospital admission was found to be significant in those children who did not develop clinical polyneuropathy, 38.5% of diphtheria survivors had abnormal nerve conduction study at six weeks of illness. Neck swelling and change in voice were significantly more common in patients with abnormal nerve conduction velocity (NCV) than in normal nerve conduction studies, 87.5% of children who had taken antibiotics before hospital admission had no clinical neuropathy, and NCV was also normal. Clinical neuropathy developed in just 20% of diphtheria patients with impaired NCV. Any child diagnosed with diphtheria should be followed for three to six months in anticipation of neurological complications. DP carries a good prognosis; hence, timely diagnosis and differentiation from other neuropathies is a prerequisite for rational management.

A Clinical Study of Complications of Diphtheria

Article

Full-text available

Mar 2022

Background: Diphtheria is a localized infection of mucous membrane or skin. To study complication of diphtheria. Methods: In this study all cases of diphtheria and all age groups which attended department of ENT. Results: It was observed that most common post diphtheria complication was myocarditis (23.00%). In neurological complication most common was palatal palsy followed by pharyngeal palsy and motor weakness. Conclusion: In order to prevent complication of the disease active immunization is customary and in order to detect the disease at early stage and for prompt treatment masses should be educated regarding the dreadful nature of the disease. Keywords: Diphtheria, Outcomes, Complication

Diphtheritic Polyneuropathy: A Rare Complication that Needs to be Acknowledged

Article

Mar 2024

Globally, diphtheria still poses a burden on public health, predominantly in developing countries. Poor vaccine coverage and boosters are the main factors in the diphtheria outbreak that should have been obliterated as the vaccine was invented a century ago. Health services are particularly burdened by diphtheria because it can lead to both short-term complications like acute airway obstruction and long-term complications like myocardial toxicity and diphtheritic polyneuropathy. Data about diphtheritic polyneuropathy is scarce, and physicians may not be aware of this condition. Herein we present the pathophysiology, clinical manifestation, diagnosis, and treatment of diphtheritic polyneuropathy.

Clinical profile and risk factors for mortality in children admitted with diphtheria: an observational study

Article

Apr 2023

Background: There is an ongoing resurgence of diphtheria infection worldwide despite a vaccine being available to prevent it for more than four decades. Objectives: To study the clinical characteristics and risk factors for mortality of diphtheria cases among children 1-12 years of age treated in our hospital from 1 April 2014 to 31 March 2021. Methodology: The data of hospitalised cases of childhood diphtheria from 1 April 2014 to 31 March 2019 were retrospectively analysed from the medical records department of our hospital. All hospitalised children with diphtheria from 1 April 2019 to 31 March 2021, were prospectively studied. All categorical variables were expressed as proportion/percentage and all continuous variables were expressed as median with interquartile range (IQR). Risk factors for morbidity and mortality were analysed and tested for significance. Unadjusted odds ratio (OR) was calculated and significant variables were subjected to multivariate logistic regression. Results: Of the 58 children with diphtheria, 62% were lab-confirmed, most cases (45%) were between 5 and 9 years of age. Majority (57%) were completely immunised as per the national immunisation schedule. Fever (97%) was the most predominant clinical symptom. The classical diphtheria pseudo membrane was identified in all. Respiratory failure was the most predominant complication, followed by myocarditis and acute kidney injury. The case fatality rate was 8.6%. Conclusion: Diphtheria cases were seen among children 5-9 years of age more commonly. Infection requiring hospitalisation was seen in vaccinated children too. No atypical manifestations were observed. Complications of the disease adversely affected the overall survival.

Tetanus, diphtheria and other toxin-producing bacterial infection of central nervous system

Chapter

Jan 2024

Diphtheria and Hearing Loss

Chapter

Nov 2023

Diphtheria is a serious bacterial infection that is the result of the causative bacterium Corynebacterium diphtheriae. Hippocrates first announced diphtheria in the fifth century B.C., and C. diphtheriae was described in 1882 [1]. Although rare, it has been reported that other Corynebacterium species (C. pseudotuberculosis, C. hemolyticus, and C. ulcerans) can cause a similar disease [2]. Clinical features vary from mild to severe. Corynebacterium diphtheriae frequently causes respiratory diseases like membranous nasopharyngitis, obstructive laryngotracheitis, or bloody nasal discharge. Less frequently than respiratory tract disease, diphtheria also causes cutaneous, conjunctival, vaginal, or otic involvements [3].

Clinical Profile and Outcome in Children with Post Diphtheritic Paralysis in a Tertiary Care Hospital in Southern India Iran

Article

Full-text available

Jan 2022

Objectives Post-Diphtheritic Paralysis (PDP), one of the most severe complications of diphtheria, is caused by exotoxin of Corynebacterium diphtheria. This study was planned since there has been a resurgence of diphtheria in India in recent years due to a number of epidemiological factors. Materials & Methods Thirty-five children with PDP were studied in a tertiary care hospital in Southern India. Result Neurological complications occurred in 38.5% of 91 patients with faucial diphtheria. Of the patients, 13 (37.1%) were unimmunized, 12 (34.3%) were partially immunized, two (5.7%) were completely immunized, and eight (22.6%) had unknown status. Isolated bulbar palsy and bulbar palsy followed by limb weakness were seen in 20 (57.1%) and 15 (42.9%) of the patients, respectively. The first symptoms of PDP occurred 5-34 days after the onset of local diphtheria infection. Eleven (31.4%) out of the 35 patients had received antitoxin between days 5-7 of illness. Ventilation-dependent respiratory failure occurred in three (8.6%) patients with PDP. Nine (25.7%) patients had evidence of co-existent myocarditis, while myocarditis with renal failure was seen in two (5.7%) patients. Four (11.4%) patients died, three from severe cardiomyopathy and one from aspiration. Demyelinating neuropathy was noted in 64% of the patients. Children with bulbar palsy recovered in 4-7 weeks, while limb symptoms improved in 6-17 weeks. Conclusion PDP should be considered in any child presenting with bulbar palsy/quadriparesis following previous history of fever/sore throat. Awareness and availability with timely administration of ADS within 48 hours are essential to reduce PDP, as antitoxin seems ineffective if administered after the second day of diphtheritic symptoms.

Clinical Profile and Outcome in Children with Post Diphtheritic Paralysis in a Tertiary Care Hospital in Southern India Iran

Article

Full-text available

Jan 2022

Diphtheria in Children- A Clinical Profile of Cases during an Outbreak in Kerala, India

Article

May 2022

Introduction: Diphtheria is an acute potentially fatal infectious disease caused by the toxigenic strains of Corynebacterium diphtheriae. Acute respiratory obstruction, toxic myocarditis and neurologic weakness are the most important complications of diphtheria. The clinical presentation and severity of diphtheria vary in immunised and non immunised children. Early diagnosis and prompt treatment including administration of diphtheria antitoxin and antibiotics minimise mortality. Aim: To observe the changing trends in the clinical presentation of diphtheria during the 2016 outbreak and its association with immunisation status and antitoxin administration. Materials and Methods: This longitudinal prospective study was conducted among children admitted to Government Medical College, Kozhikode, Kerala, a tertiary care centre with a diagnosis of diphtheria during January 2016 to December 2016. Details of socio-demographic data, clinical presentation, investigations, immunisation status, treatment and complications were collected using a semi-structured performa. These children were managed by an interim guideline provided by the state authorities. They were followed-up for 3 months i.e. till March 2017. The data was analysed using Statistical Package for Social Sciences (SPSS), version 18.0. Results: Among 76 children, 62(81.6%) were from Malappuram and Kozhikode districts, which have relatively low immunisation coverage. Most admissions were in July 2016. Majority 58 (76.3%) of children belonged to Muslim community. The mean age was 8.1 years with male to female ratio 1.53:1. Most of the children 47 (61.8%) were unimmunised or partially immunised. Cultures were positive for C. diphtheriae in 20 children. Complications were noted in 36 children, which included asymptomatic myocarditis in 31, symptomatic myocarditis in one, palatal palsy in nine, loss of accommodation in four and distal weakness in five. Only one child who received antitoxin within 72 hours of disease onset developed neurological complications. Complications were common in children who received less than minimum three doses of diphtheria vaccines compared to those who received three or more doses (54% vs. 44%). There was no mortality. Conclusion: There was an upward shift in age of affected children. Neurological complications were significantly less in those who received antitoxin within 72 hours of disease onset. Regular monitoring helped to detect asymptomatic myocarditis. The outbreak highlighted the need to improve awareness about diphtheria and better vaccination coverage, especially in older children.

Clinical Profile and Outcome in Children with Post Diphtheritic Paralysis in a Tertiary Care Hospital in Southern India Iran

Article

Full-text available

Apr 2022

Vykuntaraju Kammasandra Nanjundagowda

Objectives: Post-Diphtheritic Paralysis (PDP), one of the most severe complications of diphtheria, is caused by exotoxin of Corynebacterium diphtheria. This study was planned since there has been a resurgence of diphtheria in India in recent years due to a number of epidemiological factors. Materials & methods: Thirty-five children with PDP were studied in a tertiary care hospital in Southern India. Result: Neurological complications occurred in 38.5% of 91 patients with faucial diphtheria. Of the patients, 13 (37.1%) were unimmunized, 12 (34.3%) were partially immunized, two (5.7%) were completely immunized, and eight (22.6%) had unknown status. Isolated bulbar palsy and bulbar palsy followed by limb weakness were seen in 20 (57.1%) and 15 (42.9%) of the patients, respectively. The first symptoms of PDP occurred 5-34 days after the onset of local diphtheria infection. Eleven (31.4%) out of the 35 patients had received antitoxin between days 5-7 of illness. Ventilation-dependent respiratory failure occurred in three (8.6%) patients with PDP. Nine (25.7%) patients had evidence of co-existent myocarditis, while myocarditis with renal failure was seen in two (5.7%) patients. Four (11.4%) patients died, three from severe cardiomyopathy and one from aspiration. Demyelinating neuropathy was noted in 64% of the patients. Children with bulbar palsy recovered in 4-7 weeks, while limb symptoms improved in 6-17 weeks. Conclusion: PDP should be considered in any child presenting with bulbar palsy/quadriparesis following previous history of fever/sore throat. Awareness and availability with timely administration of ADS within 48 hours are essential to reduce PDP, as antitoxin seems ineffective if administered after the second day of diphtheritic symptoms.

Detection of Diphtheritic Polyneuropathy by Acute Flaccid Paralysis Surveillance, India

Article

Full-text available

Sep 2013

Diphtheritic polyneuropathy is a vaccine-preventable illness caused by exotoxin-producing strains of Corynebacterium diphtheriae. We present a retrospective convenience case series of 15 children (6 girls) <15 years of age (mean age 5.2 years, case-fatality rate 53%, and 1 additional case-patient who was ventilator dependent at the time of last follow-up; median follow-up period 60 days) with signs and symptoms suggestive of diphtheritic polyneuropathy. All cases were identified through national acute flaccid paralysis surveillance, which was designed to detect poliomyelitis in India during 2002-2008. We also report data on detection of diphtheritic polyneuropathy compared with other causes of acute flaccid paralysis identified by this surveillance system.

Diphtheritic polyneuropathy

Article

Full-text available

Jan 2000

Post-diphtheritic neuropathy: A clinical study in paediatric intensive care unit of a developing country

Article

Nov 2012
TROP DOCT

A retrospective study was done on 48 consecutive patients with clinical diagnosis of post-diphtheritic neuropathy admitted to the paediatric intensive care unit of tertiary care hospital in North India between January 2008 and December 2010 to study the clinical profile of post-diphtheritic neuropathy in children. The case records were reviewed and information regarding personal details, clinical features, recovery parameters and outcome was recorded using a predesigned proforma. Median age was 4.25 years. All cases were unimmunized. Median latency period was 15 days. Of the children, 52% had palatal palsy whereas 48% had limb weakness initially. Median duration of progression of weakness was five days. Limb muscle weakness was present in 94%. Respiratory muscles were involved in 85.4% cases and 60.4% required mechanical ventilation, while 14.6% had fatal outcome and 10.4% had hypoxic neurological injury. Boys were affected more. Median duration of latency was shorter; muscle weakness, progression and recovery were faster as compared with observational studies in adults.

Diphtheritic neuropathy, an analysis based on muscle and nerve biopsy and repeated neurophysiological and autonomic function tests

Article

Aug 1989

A patient with diphtheritic neuropathy was investigated with repeated tests of parasympathetic and sympathetic vasomotor and sudomotor functions for one year after the onset of symptoms. Somatic nerve function was tested with nerve conduction studies and an index based on ten variables was used to follow the course of the neuropathy. Sural nerve and anterior tibial muscle biopsies were performed. A severe but shortlasting impairment of the parasympathetic vagal reflex arc was found. The recovery of this function paralleled the clinical course. Sympathetic functions were normal. The neurophysiological variables of somatic nerve function showed signs of a mainly demyelinating mixed sensory/motor neuropathy. The recovery of these variables was slow. The nerve and muscle biopsies demonstrated mild changes consistent with a mixed, demyelinating, non-inflammatory neuropathy.

Neurological complications of diphtheria

Article

Aug 1974

Summary Eightysix children with neurological complications of diphtheria are reported. The disease accounted for nearly 20.9% of the treated cases in the S.C.B. Medical College Hospital, Cuttack. A majority of the cases of diphtheritic paralysis occurred between the age of 2 to 5 years and there were more children from the rural than from the urban areas. Palatal paralysis was a very common neurological complication which occurred alone or in association with other types of paralyses. The different neurological manifestations did not follow any definite order or pattern. The mortaiity was more in diaphragmatic and palatal paralysis. Those who recovered did not show any residual damage.

Diphtheria toxin inhibits the synthesis of myelin proteolipid and basic proteins by peripheral nerve in vitro

Article

Feb 1973

— Diphtheria toxin (DT) did not produce measurable degradation of myelin proteins or sulphatide in sciatic nerves of chick embryos after incubation in vitro for 4 h. In contrast, DT inhibited the in vitro incorporation of L-[U-14C]leucine into myelin proteins by the nerves after a delay of 1 h. Separation of the myelin proteins by SDS-polyacrylamide gel electrophoresis indicated that the synthesis of Wolfgram proteins and proteins not entering the gel was inhibited by 21–22 per cent, whereas synthesis of myelin proteolipid and basic proteins was inhibited by 79–88 per cent. Incorporation of 35SO4 into myelin [35S]sulphatide was also inhibited by DT after a delay of 2 h. The inhibition of [35S]sulpha-tide incorporation into myelin caused by DT differed from that observed with puromycin in that it did not depend on depletion of an intracellular transport lipoprotein. Instead, the inhibition seemed to be secondary to the decreased synthesis of myelin proteolipid and basic proteins.

Diphtheritic polyneuropathy: A clinical study and comparison with Guillain-Barré syndrome

Article

Nov 1999

Clinical features of 50 adults with diphtheritic polyneuropathy (DP) were studied in Riga, Latvia and compared with 21 patients with Guillain-Barré syndrome (GBS). Neurological complications occurred in 15% of patients admitted to hospital with diphtheria and usually after severe pharyngeal infection. Bulbar dysfunction occurred in 98% of patients with DP and only 10% of patients with GBS. Limb weakness was mild or absent in 30% of patients with DP. Ventilation dependent respiratory failure occurred in 20% of patients with DP. The first symptoms of DP occurred 2-50 days after the onset of local diphtheria infection. Neurological deterioration in DP continued for a median of 49 (range 15-83) days and improvement started 73 (range 20-115) days after onset. In 66% of patients with DP, the neuropathy was biphasic with a secondary worsening after 40 days. By contrast patients with GBS worsened for only 10 days on average (range 2-28 days) and improved after 21 (range 4-49) days. Eight patients with DP died, four from severe cardiomyopathy and four from multiple diphtheritic organ failure. Prolonged distal motor latencies (DMLs) were common to both DP and GBS, and more pronounced than motor conduction slowing. Limb symptoms continued after 1 year in 80% of the patients with DP, 6% were unable to walk independently, but independent respiratory and bulbar function had returned in all survivors. By comparison no patients with GBS died and none were severely disabled after 1 year. No death, in patients with DP occurred after antitoxin on days 1 or 2 after onset of diphtheria symptoms, whereas identical rates of death and peak severity of DP were seen both in those who received antitoxin on days 3-6 and those who did not receive it at all. Diphtheric polyneuropathy is much more likely than GBS to have a bulbar onset, to lead to respiratory failure, to evolve more slowly, to take a biphasic course, and to cause death or long term disability. Antitoxin seems ineffective if administered after the second day of diphtheritic symptoms.

Resurgence of diphtheria in the newly independent states of the former Soviet Union: A reminder of risk

Article

Nov 1999

AF Hann

The introduction of universal childhood immunisation programmes with diphtheria toxoid in the 1950s has led to virtual elimination of diphtheria from most developed countries. In the past, the highly contagious acute infectious disease caused by toxigenic strains of Corynebacterium diphtheriae had endangered in particular children of preschool age. Given a case fatality rate of 5% to 10%, diphtheria had been considered one of the most serious communicable diseases of childhood. The practice of routine mass immunisation of children resulted in a dramatic decline of incidence and mortality from diphtheria in many European countries, including the former Soviet Union where, by 1976, the incidence rate had fallen to 0.08 per 100 000 population.1 In 1989, a World Health Organisation (WHO) meeting endorsed a recommendation that envisaged full …

The Pathology of Diphtheria

Article

Mar 2000

Diphtheria is an acute, communicable disease caused by Corynebacterium diphtheriae. The disease is generally characterized by local growth of the bacterium in the pharynx with pseudomembrane formation or, less commonly, in the stomach or lungs; systemic dissemination of toxin then invokes lesions in distant organs. Acute disease of the upper respiratory tract usually involves one or more of the following: tonsillar zones, larynx, soft palate, uvula, and nasal cavities. A recent epidemic in Russia emphasized the role of vaccination in reducing disease in children and adults.

Diphtheritic polyneuropathy: Clinical analysis of severe forms

Article

Oct 2001
ARCH NEUROL-CHICAGO

Diphtheritic polyneuropathy (DP) is a dangerous complication of diphtheria, especially its severe forms with bulbar, respiratory tract, and circulatory disturbances. However, the clinical picture of severe forms of DP is practically unknown. To investigate the clinical features and peculiarities of the course of severe forms of DP. Thirty-two patients with severe forms of DP. The first symptoms of DP developed in most patients 3 to 5 weeks after the onset of diphtheria. The cranial nerves were involved in all patients, most frequently nerves IX and X (32 patients); VII (28 patients); III, IV, and VI (27 patients); and XI (27 patients). One third of the patients had quadriplegia. The remaining patients had quadripareses. Of the 32 patients, 24 underwent artificial ventilation. All patients had sensory signs, proprioceptive more often than superficial. Autonomic disturbances were observed also in all patients. Only 2 of the 32 patients died. A direct indication for tracheotomy and artificial ventilation in patients with DP is a decrease of the vital capacity of the lungs below the traditional 16 mL/kg body weight or the development of the paralytic closure of the larynx against the background of the increasing weakness of the respiratory muscles. Characteristic of severe forms of DP is the phenomenon of the oppositely directed change in the neurological symptoms in the second month of the disease: the restoration of the function of the cranial nerves against the background of the further increase of the motor disturbances in the extremities and trunk. Special attention and care should be taken of patients during the period of the appearance of the episodes of vascular collapses-between the fourth and seventh weeks of DP.