Transcript
Page 1: Neurological disorders in complex humanitarian emergencies and natural disasters

NEUROLOGICAL PROGRESS

Neurological Disorders in ComplexHumanitarian Emergencies

and Natural Disasters

Farrah J. Mateen, MD

Complex humanitarian emergencies include the relatively acute, severe, and overwhelming health consequences ofarmed conflict, food scarcity, mass displacement, and political strife. Neurological manifestations of complexhumanitarian emergencies are important and underappreciated consequences of emergencies in populationsworldwide. This review critically assesses the existing knowledge of the range of neurological disorders thataccompany complex humanitarian emergencies and natural disasters in both the acute phase of crisis and the ‘‘longshadow’’ that follows.

ANN NEUROL 2010;68:282–294

Complex humanitarian emergencies occur when civil-

ian populations experience immediate, severe, and

overwhelming health consequences. Armed conflict, mass

displacement, and food scarcity are major threats to pop-

ulations worldwide.1 Although rarely considered, neuro-

logical manifestations are important components of com-

plex humanitarian emergencies.

This review explores the neurological manifestations

of complex humanitarian emergencies, where docu-

mented, from more than 100 countries around the

world. Whereas historical perspectives of neurological

disease and emergencies have focused on combatants,

modern emergencies are increasingly experienced by

unarmed civilians. Armed conflict today is more often

protracted, intrastate, fought by irregular groups, and

marred by targeted violence toward civilians.2 Civilians

are now the most likely group to experience both mor-

bidity and mortality in times of warfare.

People in low-income and middle-income coun-

tries are especially at risk for health consequences of

emergencies. Existing health services may be inadequate

or absent and a priori health status may be poor. How-

ever, neurological consequences of emergencies are not

confined to people in resource-poor regions. Emergen-

cies and disasters are experienced in all parts of the

world, leading to a heightened need for neurological

services and increasing the already-high burden of neu-

rological disease.

The aim of this review is to provide a topical

understanding of neurological manifestations in times of

armed conflict, mass displacement, natural disasters, fam-

ine, and torture experienced over the past generation

(1985 to present). The first goal is to concentrate atten-

tion on the real experiences of people who suffer neuro-

logical disorders in crisis settings. The second is to pro-

vide a useful framework for policymakers, aid workers,

and relief organizations to include neurological disorders

in the priority setting arena.

Because neurological disorders in times of crisis can

be both acute and chronic, each type of humanitarian

emergency will be considered in relation to: (1) the more

immediate manifestations of neurological injury that

accompany a disaster, relevant to relief workers and res-

cue agencies; and (2) the chronic neurological disorders

of survivors; ie, those disorders that persist in the ‘‘long

shadow’’ of crisis in the months to years that follow basic

relief efforts. Although psychiatric disorders have a com-

plex interplay with the experience of neurological disor-

ders, this review will focus solely on those disorders tradi-

tionally considered as neurological.

View this article online at wileyonlinelibrary.com. DOI: 10.1002/ana.22135

Received Feb 13, 2010, and in revised form May 26, 2010. Accepted for publication Jun 18, 2010.

Address correspondence to Farrah J. Mateen, Room E8527, 615 N. Wolfe Street, Baltimore, MD. E-mail: [email protected]

From the Department of International Health, Bloomberg School of Public Health, The Johns Hopkins University, Baltimore, MD.

282 VC 2010 American Neurological Association

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Armed Conflict

Acute PhaseTraumatic injuries to the brain, spinal cord, plexus, and

peripheral nerves are well-documented during armed

conflict (Table 1). Damage may be direct, due to violent

warfare between combatants or combatants and civilians,

or indirect, due to exploding ordinances postconflict.

BRAIN INJURY. Traumatic brain injury is the most

commonly documented neurological consequence of

armed conflict and exceeds the rate of other injuries,

including amputations, in many countries. Between 2003

and 2007, Military Health Services of the United States

reported more than 43,000 new diagnoses of traumatic

brain injury related to Operation Enduring Freedom,

Operation Iraqi Freedom, and unaffiliated wars. The esti-

mated cost in 2009 was 100 million $US for direct care

with an additional 10 million for related prescription

drug costs.3

Although nearly all reports of traumatic brain

injury derive from combatants and veterans, several stud-

ies from Afghanistan, Bosnia, Croatia, Iran, Iraq, Israel,

and Lebanon have looked at civilian injuries. In recent

years, pediatric traumatic brain injury,4,5 self-inflicted

head injury,6 and secondary neurological consequences of

traumatic brain injury, including intracranial infection,7–9

post-traumatic epilepsy,10,11 and chronic headache,12,13

have received greater attention. Secondary neurological

complications may exist in up to 35% of penetrating

brain injuries.7 Likewise, the consequences of mild trau-

matic brain injury are increasingly recognized.14

Survival following traumatic brain injury varies

widely in recent reports. In Croatia (1991–1994), the

mortality rate at a frontline hospital was 46%15 and in

Bosnia and Herzegovina (1994–1995) it was 37%,16

whereas in U.S. Field Hospitals (1991) in Operation De-

sert Storm, the mortality rate was approximately 5%.17

The first 2,000 wartime hospital admissions of children

in Iraq and Afghanistan were reported in 2008.4 Case fa-

tality rates of head injury far exceeded any other cause of

admission and accounted for 30% of all deaths among

admitted children. Prehospital mortality in Afghan chil-

dren was considered high and may have deflated the real

pediatric case fatality from war. A lack of standard defini-

tions for level and type of injury prevents robust compar-

isons between locations and cohorts.

In current wars, blast injuries are the most common

form of head injury and may be due to rocket and mor-

tar shells, artillery, booby traps, aerial bombs, improvised

explosive devices, landmines, or rocket-propelled gre-

nades.18 Antipersonnel landmines have received concen-

trated attention in recent years with the aim to ban their

TABLE 1: Reported Neurological Manifestations by

Category of Emergency (1985–2009)

Humanitarian

Emergency

or Event

Neurological Manifestations

Documented in Association

with the Emergency

Natural disasters Seizures

Pseudoseizures

Head and spinal cord injury

Peripheral nerve and brachial plexus injury

Ischemic and hemorrhagic stroke

Worsened symptoms of dementia

Vertigo

Delayed vaccination leading to

polio outbreaks

Meningitis from contaminated

anesthetic storage

War/conflict Head and spinal cord injury

Post-traumatic CNS infection

Neuropathic pain

Epilepsy, post-traumatic

Headaches, chronic, post-traumatic

Peripheral nerve injury and neuromas

Multiple sclerosis exacerbations

Memory loss

Meningococcal meningitis outbreaks

Japanese encephalitis outbreaks

Tuberculous meningitis outbreaks

Polio outbreaks

Measles outbreaks

Cerebral malaria

Long-term: brain tumors

Food scarcity Epilepsy

Toxicity from antiepileptic drugs

Myeloneuropathy

Peripheral neuropathy

Optic neuropathy

Sensorineural deafness

Wernicke’s encephalopathy

Seasonal ataxic syndrome

Strachan’s syndrome

(B-complex vitamin deficiency)

CNS ¼ central nervous system.

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use worldwide. Landmines can be found in more than

70 countries with a predominant effect on civilian youth

in low-income and middle-income countries.19 In 2009,

20 people were maimed or killed by antipersonnel land-

mines on a daily basis.20 Although a significant fraction

of landmine-related injuries are fatal (50–55%),21 acute

head and spinal cord injury has been reported.22,23 In Sri

Lanka, where injuries were recorded by body part, 16%

of landmine injuries occurred above the neck.24 Children

are likely to be more affected by head injury than adults

because they are more likely to pick up landmines than

adults and have their vital organs closer to the point of

impact.25,26

SPINAL CORD INJURY. Spinal cord injury is a major

cause of severe disability during armed conflict.27 In

some recent conflicts, civilian mortality rates from spinal

cord injury and its complications show only modest dec-

rements from the nearly 80% mortality reported by Har-

vey Cushing in 1927.28 In recent years, spinal cord

injury has been reported from Afghanistan, Senegal, Bos-

nia, Croatia, Iraq, Israel, and Lebanon. A study in an

evacuation hospital in Croatia revealed injuries due to

projectiles from automatic rifles, sniper fire, and frag-

ments of explosive devices. The average age was 39 years

old in civilian causalities but 28 years old in military cas-

ualties.29 Permanent paralysis or death was found in

nearly half of spinal cord injury patients in 2 Croatian

referral hospitals.29,30 Six years postconflict, there was no

significant change in the number of rehabilitation beds,

facilities, or professionals in Croatia.31 A referral hospital

for Afghan victims of war in Pakistan saw more than

1,700 patients for spinal cord injury between 1983 and

1996.32 In Afghanistan in 2003, a study of 311 residents

of Kabul and Herat found acute care for spinal cord

injury survivors was ‘‘practically nonexistent,’’ with a high

prevalence of pressure sores and recurrent urinary tract

infections among spinal cord injury survivors. In Kabul,

63% of all spinal cord injuries in an International Com-

mittee of the Red Cross spinal cord injury program were

attributed to war.32 In Africa, reports on spinal cord

injury during armed conflicts are limited. A referral hos-

pital in Senegal found that less than half of patients with

spinal cord injury experienced partial or complete recov-

ery, citing a special need for immediate and multidiscipli-

nary management of spinal cord injury patients.33

PERIPHERAL NERVE INJURY. Peripheral nerve injuries

are often more common than traumatic brain and spinal

cord injuries during conflict34 but are comparatively

understudied. Peripheral nerve and plexus injury can

result from direct trauma, chemical warfare, and long-

term damage from unhealed fractures. The Belgrade

Military Medical Academy managed 3,091 missile-

induced peripheral nerve injuries from 1991 to 1995.35

A total of 713 patients (99% male) were reported with

peripheral nerve injuries due to war in Croatia and Bos-

nia and Herzegovina. The majority had single-nerve

lesions with the peroneal (21%) and ulnar (20%) being

the most common.36 In U.S. Army casualties of the Per-

sian Gulf Conflict, the most common nerves affected in

one report were median (18%), peroneal (16%), ulnar

(12%), and radial (11%)34; however, when considering

10-year outcomes, there was no significant difference in

the rate of peripheral neuropathy between deployed and

nondeployed Gulf War veterans.37

Peripheral nerve injuries have a long-term relation-

ship to pain among survivors of armed conflict. High-ve-

locity missile injuries comprised more than 75% of

reported cases in a meta-analysis of ‘‘causalagia’’ in

2003.38,39 In Serbia, 15% of peripheral nerve injuries

caused by missile were considered painful.35 Multimodal-

ity treatment including early nerve repair40 and regional

anesthesia41 have been studied more thoroughly as a result.

Chronic PhaseIn times of conflict, health systems fall into disrepair and

persons with chronic diseases cannot access health serv-

ices. Chronic diseases are often not priorities in resource-

poor regions, even before an armed conflict arises. In

Nicaragua, prolonged conflict between 1983 and 1987

led to rampant inflation of service fees, targeted killings

of health care professionals, and weakening of basic

health services.42 In Iraq, doctors left Baghdad due to

threats, kidnappings, violent events, and violent deaths,

leading to a peak loss of 22% of specialists in 2007.43 In

Sierra Leone, the 1 neurologist in practice during the

civil war moved out of the country.44

In Kosovo in 1999, high numbers of deaths were

reported to be due to renal disease, ischemic heart dis-

ease, and diabetes,45 but the neurological causes of death

were not specifically reported. A similar situation likely

occurred in Chechnya and Ingushetia,46 demonstrating

the need to emphasize chronic disease care when human-

itarian emergencies occur in developed regions. A combi-

nation of targeted political civilian violence, declining

access to health care, lack of fuel, and mistrust of existing

health services can lead to a predominance of untreated

chronic conditions. In contrast, malnutrition and infec-

tious diseases are the usual causes of death seen during

emergencies in developing countries.47

The theory that stress may increase the rate of mul-

tiple sclerosis (MS) exacerbations has been posited. ‘‘War

stress’’ would therefore be a special concern among

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people with diagnosed MS. A study of 156 patients with

relapsing-remitting MS patients in Israel48 found a 3- to

18-fold increase in relapse rate during a 33-day period of

war as compared to the rate a year earlier when no war

occurred. Patients who reported high levels of distress

due to rocket attacks, perceived death threats, or home

evacuation were more likely to have an attack. A similar

study in Lebanon49 found patients with clinically-definite

MS have both increased rates of relapse during war and a

higher number of gadolinium-enhancing lesions on mag-

netic resonance imaging of the brain.

United States veterans of World War II have been

noted to have increased rates of MS compared to the av-

erage U.S. population, with a marked excess seen in all

age-sex groups.50 Long-term prisoners-of-war from the

Far East, however, were not noted to have an excess of

MS or any other neurodegenerative disease after 46 years

of follow-up.51

Survivors of landmine injury may experience mem-

ory loss,52 painful neuromas of the amputated stump,53

postamputation pain,54 phantom limb phenomena,55

and significant locomotor disability.56 Rehabilitation

services for landmine survivors reach less than 5% of

people with disability in developing countries.57

Long-term follow up for neurological mortality was

studied among 621,902 Persian Gulf Veterans (1991–

1992) and 746,248 nonveterans.58 No neurological mor-

tality was associated with Gulf War service, including

death due to motor neuron disease, Parkinson’s disease,

multiple sclerosis, or brain tumors; however, veterans

potentially exposed to nerve agents at Khamisiyah, Iraq,

and to smoke from oil-well fires had a higher risk of fatal

brain cancer.

Neurological Disorders in Refugees andInternally Displaced Persons

The United Nations recognizes 26 million refugees and

16 million internally displaced persons.59 Refugee camps

are often not temporary arrangements, and persist for

years and decades with inadequate health resources and

vulnerable populations. Displaced persons are at height-

ened risk of neurologic infections due to overcrowding,

poor sanitation, and limited access to health care in some

settings. Chronic neurological diseases may also be im-

portant causes of illness but have not been reported in

this group.

Meningococcal MeningitisRefugees in the meningitis belt—a 600-km area stretch-

ing from Mali to Sudan—include Sudanese and Somali

refugees in Ethiopia, Ethiopians and Chadians in Sudan,

and internally displaced Sudanese, and comprise a total

of 3.5 million displaced persons or more.1 Vaccination

rates for meningococcal disease are particularly low in

sub-Saharan Africa. For example, in southern Sudan,

99% of the population is not vaccinated,60 although each

quadrivalent meningococcal vaccine costs between 20

and 25 cents.61 A recent outbreak in Chad in spring

2010 led to mass vaccination, but efforts in Darfur have

been hampered by expulsion of core relief agencies.62

Outbreaks of meningococcal meningitis (�15 cases

per 100,000 people per week for 2 weeks) can occur

multiple times annually in the ‘‘meningitis belt.’’63 Epi-

demic meningitis, due to N. meningitides among refu-

gees, has been frequent enough to prompt guidelines

from the World Health Organization on the appropriate

interventions to address an outbreak.64 Minimum

requirements include an appropriate case definition and

adequate organism identification (Table 2).65–68

In 1992, in the lowlands between India and Nepal,

73,500 Bhutanese refugees were monitored in 6 refugee

camps.69 A working case definition of suspected meningi-

tis or encephalitis was ‘‘fever, with new seizures, confusion,

and/or coma in a patient older than 5 years with a blood

smear that tested negative for parasites.’’69 An outbreak of

meningoencephalitis was reported in 116 patients (case fa-

tality rate of 13%) but testing was unavailable to deter-

mine the etiologic agent, deterring vaccination against

both meningococcus and Japanese encephalitis.

Infectious disease was a major cause of registered

and unregistered deaths among Iranian prisoners of war

in Iraqi detention camps between 1980 and 1990.

Although meningitis was among the most common rec-

ognizable causes of death, the exact prevalence was

unclear.70

MalariaMalaria is a significant cause of disease among refugees

and combatants in countries where the disease is

endemic. In 2008, there were 90,817 confirmed cases of

malaria reported in refugees in Chad, Ethiopia, Kenya,

Rwanda, Sudan, Tanzania, and Uganda.71 Malaria also

remains a problem outside of sub-Saharan Africa in Hai-

tian and Afghan refugees and Iraqi civilians.

The rate of cerebral malaria among refugees has

not been recorded. In military personnel, Brazilian troops

in Angola were observed for 6 months in 1995–1996.

Among 439 nonimmune troops, there were 78 cases of

malaria (attack rate 18%) and 3 deaths due to cerebral

malaria.72 Among U.S. troops in Somalia deployed for 6

months, there were 48 cases of malaria, including 1 case

of cerebral malaria.73 Headache with fever was a major

presenting feature of all forms of malaria (96% of cases)

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among U.S. troops in Somalia73 and Sierra Leone

(79%).74 If cerebral malaria complicates even 0.5% of all

malaria cases, a conservative estimate of the number

affected can be made. For example, during a malaria epi-

demic in Burundi (October 2000–March 2001), 2.8 mil-

lion people had malaria,75 potentially yielding 14,000

cases of cerebral malaria alone.

PoliomyelitisArmed conflict has affected poliomyelitis (polio) eradica-

tion efforts in more than 20 countries across Eastern

Europe, Central and South America, Asia, and Africa in

the past 25 years (Fig 1). A variety of factors are to

blame, including reduced access to susceptible children

in insecure locations, dysfunctional medical laborato-

ries,76 lost medical records,77 overcrowding among dis-

placed persons, and kidnapping and death of vaccination

team employees.78–80 For example, in Bajaur, Pakistan,

an official was killed by a roadside bomb after returning

from a meeting with tribal elders to try to dispel myths

that polio vaccination led to sterilization.78

In 2008, the World Health Organization stated that

much of Afghanistan remained polio-free, with the excep-

tion of the Southern Region, where conflict is prominent,

and more than 90% of the region was unreachable due to

security restrictions. As few as 13% of children in this

region received 3 doses of oral polio vaccine as compared

to nearly 70% in the Central and Eastern regions.79 A sur-

vey of youth in Kandahar, Afghanistan, found more dis-

ability due to poliomyelitis than any other cause, including

landmines and other war-related injuries.81

West Darfur suffered a resurgence of polio in 2004

when approximately half of 1.2 million displaced persons

could not be vaccinated by the United Nations and aid agen-

cies.82 Nonetheless, as one of the longest running global

health programs, the polio eradication campaign has demon-

strated that vaccination of children can occur in the absence

of formal truces. So-called ‘‘Days of Tranquility’’ have been

negotiated in more than 7 war-torn countries in order for vac-

cinators to reach children.83,84 This has led to immunization

days in the conflict zones of Sudan (reaching 1 million previ-

ously unvaccinated children),84 Angola (2002; 3.1 million

children),85 and Somalia (1998; 1.4 million children).84

MeaslesSevere and devastating measles outbreaks have occurred in

the emergency setting, and are exacerbated by malnourish-

ment. Delayed and incomplete vaccination has been a

major cause of illness in Sudan,75 Somalia,75 Democratic

Republic of Congo,75 Afghanistan,86 Bosnia,87 Ethiopia,88

Guinea-Bissau,89 Iraq,86 and Sierra Leone.90 Although

children are at risk of subacute sclerosing panencephalitisTABLE2:ReportedMeningoco

ccalMeningitis

Outb

reaksin

ConflictZonesandRefugeeCamps(1990–2

008)

Year

Location

Estim

ated

Number

ofCases

Case

Fatality(%

)

Age

Group(yr)

Serogroup/

Global

AttackRate

Impact

MitigatingFactors

Durationof

Outbreak

(mo)

1999

Yam

bala,

Angola66

253

5815–29mostcommon

;

then

5–14

A:0.36%

92%

ofreportedcaseshad

no

accessto

medicalfacilities

Civilwar;econ

omicdecline;

lowhealthservicecoverage

4

1994

Gom

a,

Zaire67

348

Not

provided

All

ASpread

through

multiplecamps

War

inRwanda;active

meningitissurveillance;postoutbreak

massvaccinationandchloramphenicol

1

1990

Sarh,

Chad

68

721

7.9

(hospitalized

patients)

Meanage,12

AMajor

risk

factor

for

dyingisdelay

in

reachinghospital

Massvaccination2yr

earlier

(estim

ated

coverage

66%)

did

not

preventepidem

ic

Not

provided

(>1)

1994

Northern

Uganda6

5

291

13.3

Highest

in15–29

A:0.30%

Spread

tolocalUgandan

inhabitants

from

Sudaneserefugees;epidem

icmay

havestartedin

campreception

center

Massvaccinationcampaign

reducedim

pact

afteroutbreak;new

refugeeinfluxledto

new

outbreak;follow-upvaccination

mop-upcampaign

12

Add

itional

outbreaksof

meningococcal

meningitisoccurred

inDem

ocraticRepublicof

Con

goin

2002

and8provincesin

Rwanda,7provincesof

Burundi,andKibon

do,

Tanzania

in2003.75

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286 Volume 68, No. 3

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later in life, this has been reported rarely,91 perhaps

because the fatality rate of measles in the refugee setting is

up to 8 times higher than in stable populations, leaving

fewer survivors overall.

TuberculosisAlthough tuberculosis (TB) is an important problem

among refugees, the neurological manifestations of tuber-

culosis in complex humanitarian emergencies are essen-

tially unreported. In the largest Kampuchean refugee camp

in Thailand, spinal and meningeal presentations accounted

for 6% and 2%, respectively, of all 629 TB cases.92 TB was

also a concern in the conflict regions of south Sudan in

2003 where spinal TB was observed in 18% and menin-

geal TB was observed in <1% of all forms of TB cases.93

Successful TB treatment programs have been initiated

worldwide in conflict zones, including East Timor,94 Af-

ghanistan,95 Kosovo,96 and South Sudan.93

Human Immunodeficiency VirusHuman immunodeficiency virus (HIV) transmission can

increase or decrease during times of conflict. In spite of a

growing literature on HIV infection and recognition of rape

as a war crime, neurological manifestations of HIV are unre-

ported or undiagnosed in emergency settings to date.

Natural Disasters

Acute PhaseNatural disasters include earthquakes, hurricanes, floods,

windstorms, snowstorms, volcanic eruptions, and wild-

fires. Since 1975, the number of people affected by natu-

ral disasters has risen steadily, including the number of

people injured and killed.97 Human vulnerability must

exist prior to environmental hazards, in the form of frag-

ile dwellings, overcrowding, barriers to evacuation, poor

rescue response, or simply being present in a location

where disasters may occur without warning. Neurological

injury resulting from earthquakes in the immediate pe-

riod includes traumatic spinal cord, brain, and nerve

injury, seizures, pseudoseizures, and stroke. In the longer

term, vertigo, increase in stroke incidence, exacerbation

of dementia symptoms, and resurgence of vaccine-pre-

ventable neurological diseases may be felt.

Following the 2005 earthquake in Northern Paki-

stan, between 650 and 750 spinal cord injuries

occurred98 with approximately 600 cases of paraplegia,

the highest reported number following any natural disas-

ter.99 The paucity of survivors with higher spinal cord

lesions raises the possibility that people with cervical cord

injuries did not live. In Northern Pakistan and in Bam,

Iran (2003), where 240 spinal cord injuries were noted,

the majority of victims were women. When earthquakes

occur in the early morning hours, more women may be

at home standing up and subject to falling debris.100

Neurologic injury is an important cause of mortality

in some earthquakes. In Athens, Greece (1999), brain and

spinal cord injury accounted for 57% of deaths in an au-

topsy-based study following an earthquake.101 In Sichuan

Province, China (2008), earthquake survivors with severe

traumatic brain injury had the highest risk of inpatient

FIGURE 1: Oral polio virus vaccine administration near Jalalabad City, Afghanistan. Courtesy of Cornelia Walther,Communication Specialist, UNICEF.

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death (Richter scale 8.0).102 Peripheral nerve injuries were

reported in 1999 following the Marmara earthquake in

Turkey. Compartment and crush syndromes led to appa-

rent brachial plexus degeneration whereas extremity pe-

ripheral nerve degeneration was associated with being

trapped under rubble and debris.103

Earthquakes may acutely increase the risk of ische-

mic104 and hemorrhagic stroke. Following the Noto Pen-

insula, Japan, earthquake (magnitude 6.9), the incidence

of hemorrhagic stroke increased for 1 month.105 This

may be due, in part, to the increase in blood pressure

and albuminuria that has been observed for up to 2

months postearthquake.106 In the elderly, stroke can be a

more frequent cause of mortality than coronary heart dis-

ease following earthquakes.107

Among patients with seizure disorders, the Nis-

qually earthquake (2001), 17km northeast of Olympia,

WA, was mistaken for a seizure by many patients. In

both patients with documented seizures due to underly-

ing epilepsy and patients with nonepileptic (‘‘pseudo’’)

seizures, the earthquake precipitated an increased rate of

events.108

Chronic PhaseIn the long term, other neurological symptoms emerge

following natural disasters. After a severe earthquake in

the Republic of Georgia, an increase in the number of

cases of peripheral vertigo was reported.109 In Japan, sep-

arate studies of patients with Alzheimer’s disease post-

earthquake found that 86% of patients had long-term

recollection of an earthquake,110 but may lose their abil-

ity to cope when forced to leave their homes.111 Also,

the increased incidence of stroke may outlast the initial

impact of the earthquake. In Japan, the Hanshin-Awaji

earthquake increased the risk of ischemic stroke in the

first year following the earthquake (relative risk 2.4).104

In Sri Lanka in 2005, an outbreak of Aspergillusfumigates meningitis after spinal anesthesia for cesarean

section occurred in 5 pregnant women. The contamina-

tion was blamed on inadequate storage conditions fol-

lowing a mass influx of donations post-tsunami.112

Natural disasters have also hampered vaccination

efforts. Persistent drought in arid climates affects the cold

chain of vaccine delivery, while in Sudan and India, sea-

sonal flooding makes vulnerable populations nearly inac-

cessible (Fig 2).82 Of the vaccination campaign in Sudan

in 1998, it was said ‘‘Heat, armed conflict, lack of infra-

structure, the need to reach more than 80% of the popu-

lation by air, infectious diseases, drought, and hungry

packs of hyenas were some of the obstacles to

overcome.’’113

Famine: Food Scarcity and NutrientDeficiencies

Famine has occurred throughout the world, affecting mil-

lions of people, and persists today. In the early 1990s in

Somalia and southern Sudan, average body mass index

among some adults was so low that texts beforehand had

reported them incompatible with life.47 Neurological

consequences of starvation are reported in nonemergency

settings based on small convenience samples.

Specific nutritional deficiencies can have primarily

neurological manifestations, including iodine, thiamine,

niacin, and possibly zinc and iron. Many of these have

been well documented, and a single micronutrient defi-

ciency does not necessarily occur in isolation. During

times of food scarcity, neurological disorders may develop

without warning. Drug toxicities may also occur: an iso-

lated report of higher than expected rates of phenobarbi-

tal toxicity occurred in the setting of a Zambian fam-

ine.114 The link between chronic malnutrition and

epilepsy is unclear. When studied in Africa, a correlation

between lower body mass index and living with epilepsy

has been shown but is derived from nonemergency

settings.115,116

The neurological consequences of protein energy

malnutrition have been reviewed.117 Children with both

protein energy malnutrition and its acute form, kwashi-

orkor, show brain abnormalities on magnetic resonance

imaging, including brain atrophy, cerebral edema, and

delays in cerebral myelination.117–119 Other manifesta-

tions include myelopathy and neuromuscular damage.117

Prisoners who deliberately undergo hunger strikes

in Turkey have been investigated for neurological conse-

quences. Gait imbalance, somatosensory disturbances,

truncal ataxia, nystagmus, and ophthalmoparesis were

commonly noted (hunger duration 153–282 days). Only

16% of patients could walk independently.120 In another

study,121 Wernicke’s encephalopathy or Wernicke-Korsak-

off syndrome, associated with amnesia and altered con-

sciousness, was noted in all 41 prisoners in spite of

thiamine supplementation (mean fasting time 199 days).

In regions of southern and western Nigeria in

1992–1993, an outbreak of ‘‘seasonal ataxia’’ was

reported. Symptoms included acute truncal and gait

ataxia, intention tremor, and nystagmus, usually after fin-

ishing an evening meal. More severely affected patients

experienced encephalopathy and ophthalmoplegia.122

The outbreak was blamed on the consumption of larvae

of Anaphe venata, a silkworm containing thiaminases, as

an alternative source of protein. Females were affected at

least 3 times as commonly as males, and all were consid-

ered to have low socioeconomic status. At the height of

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the epidemic, there were 1,126 admissions for seasonal

ataxic syndrome in Ikare, Nigeria (attack rate 1.87%).123

A subsequent placebo-controlled study found thiamine

supplementation significantly effective for the treatment

of seasonal ataxia. Similar outbreaks have occurred in

Tanzania.124

Wernicke’s encephalopathy, another clinical syn-

drome of thiamine deficiency, shares clinical features

with seasonal ataxia, but lacks the periodicity and meal-

response described in seasonal ataxia. Pediatric cases of

Wernicke’s encephalopathy, especially as a primarily ataxic

syndrome, are likely underdiagnosed as nutrient deficiency

and may represent chronic malnutrition states. Thiamine

deficiency was a major cause of infant mortality among

Karen refugees in Thailand in the late 1980s, accounting

for 40% of all infant deaths before supplementation.125

In Cuba, more than 50,000 people (461 per

100,000) were affected by an epidemic of neuropathy

attributed to thiamine, cobalamine, folate, and sulfur

amino acid deficiencies, even though malnutrition was

not overt. Bilateral retrobulbar optic neuropathy, sensori-

neural deafness, dorsolateral myeloneuropathy, dysphonia,

dysphagia, spastic paraparesis, distal peripheral axonop-

athy, and mixed forms were noted.126 The 1992–1993

epidemic, which did not recur, was linked to the collapse

of the Soviet Union and the ongoing embargo by the

United States.127

Torture

‘‘Torture is the systematic destruction of person, family,

neighborhood, school, work, formal and informal organiza-

tions, and nation, with the purpose of controlling a popula-

tion the state perceives to be dangerous.’’128 According to

Amnesty International, torture is practiced systematically in

more than 120 countries and experienced by one-third of

refugees.129 Torture victims can experience extreme forms of

neurological injury. Head injury was the most commonly

reported neurological manifestation in Cambodian refugees

in Thailand border camps,131 Kurds seeking asylum in the

United Kingdom,52 and South Vietnamese ex-political

detainees.132 Causes include beatings, whipping, gunshots,

stabbing, asphyxiation, prolonged suspension, submersion,

and electrocution.129,133 Neurological consequences of tor-

ture occur during the time of torture and afterward. Head-

aches, vertigo, loss of consciousness, nerve and spinal cord

injuries, wound and scar pain, muscle stiffness, muscle atro-

phy, paralysis, and hearing and vision loss have all been

reported.129,133 Neurological injury may occur from suicide

attempts among survivors,134 including children,135 who

may suffer from self-induced asphyxia or toxic effects of poi-

soning. In Sierra Leone, the arms of war prisoners were tied

with cables, leading to severe ischemic damage to the pe-

ripheral nerves. According to a physician’s report at the

time: ‘‘The damage was irreversible with the functional out-

come no better than the amputees.’’44

FIGURE 2: Immunization team crossing a river during seasonal flooding, Bihar, India. Courtesy of Dr. Devendra Khandit,National Polio Surveillance Unit, Delhi, India.

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September, 2010 289

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Conclusions

Neurological manifestations of complex humanitarian

emergencies are documented from countries around the

world. These manifestations could be addressed through

a combination of surveillance, prevention, awareness, and

treatment. Advocacy efforts require both coordination

and education in locations where few if any neurologists

exist and depend on the international community of neu-

rologists, policymakers, and other skilled workers.

Complex humanitarian emergencies are common

and changing. From 1991 to 2002, 25 countries experi-

enced complex humanitarian emergencies with a crude

mortality rate of at least 1 per 10,000 persons per day.47

In 2009, the United Nations High Commission for Ref-

ugees estimated that 42 million people remained force-

fully uprooted from their homes due to persecution or

fear of persecution.58 Thirty-five ongoing armed conflicts

are recognized by global security organizations in

2010.136,137 In the face of climate change, urbanization,

aging, and rising incomes, the experience of civilians is

different today than even a generation ago.

A policy framework to approach neurological disor-

ders in crisis depends on baseline epidemiological and

clinical knowledge. The following recommendations are

4 actions for policy that consider neurological disease.

1. Long-Term Monitoring and Surveillance ofNeurological Manifestations During CrisesConcerted efforts have not yet been made to count or

monitor neurological illnesses in complex humanitarian

emergencies or natural disasters. One successful strategy

has been the surveillance of meningococcal meningitis in

some refugee camps. Simple guidelines include the moni-

toring of overt symptoms including coma and neck stiff-

ness. These tasks do not require a neurologist, but merely

a neurological perspective. Other neurological diseases

may also benefit by monitoring and can be studied

through relatively simple and cost-effective means,

including verbal autopsy, precrisis civil registration sys-

tems, and development of valid case definitions. The rel-

ative success of identifying neurological manifestations in

some of the poorest regions of the world already suggests

that identification of the problem is a necessary first step.

2. Vaccination and Prevention of NeurologicalDisorders in Vulnerable PopulationsVaccination of large numbers of refugees in sub-Saharan

Africa has averted more serious and widespread disease in

displaced populations in recent years. Days of Tranquility

for polio vaccination have occurred on 4 continents in

spite of ongoing conflict and in the absence of formal tru-

ces. This has informally led to opening of communication

channels, negotiations among warring factions, de-mining,

and putting down weapons, success that reaches beyond

the intended achievements of vaccination alone.99,110

3. Advocacy and Awareness of the PredominantNeurological Disorders in DifferentEmergency SettingsThe knowledge that neurological disease may exacerbate

humanitarian emergencies can lead to changes that are

both lifesaving and cost-effective. Research that takes

advantage of demographic and geographic variables in

complex humanitarian emergencies can more appropri-

ately target services and resources to vulnerable popula-

tions. For example, in Afghanistan, a significant burden

of locomotor disability in war was found to be due to

polio in women while, in men, a combination of land-

mine injury and trauma was most often to blame. This

is one of the first locations to report that the etiologies

of neurological manifestations of war may differ among

civilian women and men, even though the consequences

may be similar.81 Similar studies following earthquakes

in Pakistan and Iran found that women outnumber men

in traumatic spinal cord injuries following earthquakes

but rarely in other settings.

4. Strengthening of Health Systems toEmphasize Chronic Diseases andDisability ServicesFinally, neurological disorders have an impact beyond the

acute stage that traditionally defines emergencies, and they

can persist in the months to years that follow postemer-

gency attention. Unfortunately, almost nothing is known

about the long-term survivors of neurological injuries from

modern-day emergencies and natural disasters. Perhaps not

surprisingly, in the few areas where long-term neurological

consequences of complex humanitarian emergencies are

reported, studies show that survivors on both sides of the

conflict typically continue to suffer. The lack of availability

and the relative cost of disability services in countries that

have experienced war and natural disaster—even years after

stability is regained—can be appalling.

This highlights the challenge of incorporating the

study and treatment of neurological disease into existing

health related efforts and the provision of humanitarian

aid as soon as possible. A rapid scale-up of services must

engage not just neurologists but also nongovernmental

organizations, donors, United Nations agencies, and

established rescue and relief operatives. A modern day

perspective demands a look beyond trauma care and ba-

sic relief efforts toward long-term improved security, care

of displaced persons, better education of health care

workers, and attainment of a sustainable peace. Although

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290 Volume 68, No. 3

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a variety of advocates will drive the way forward for

advancing neurological care, many of the issues raised

above can best be prioritized by neurologists. Neurolo-

gists in resourced settings know and witness the signifi-

cant treatment benefit that occurs in people with

adequate care. They are also in a position to advocate for

a more dignified existence for people in times of crisis.

Acknowledgments

I thank Drs Gilbert Burnham, MD, Robert E. Black,

MD, MPH (Department of International Health,

Bloomberg School of Public Health, Johns Hopkins Uni-

versity), and Daniel H. Lowenstein, MD (Department of

Neurology, University of San Francisco) for their helpful

comments during the preparation of this manuscript.

Dr Mateen is supported by the Sommer Scholars

Program at the Johns Hopkins University School of Pub-

lic Health and the 2010 American Academy of Neurol-

ogy Practice Research Fellowship Grant.

Potential Conflict of Interest

Nothing to report.

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