NERVOUS DISORDER

SUBMITTED TO:- BY :–

Dr. Yadnya Parvate Priyamvada Arte (BBT-1-18006)

Deepa Tiwari (BBT-1-18007)
Dhruwa Patil (BBT-1-18009)
Husaina Freeganjwala (BBT-1-18010)
Ashlesha Gadge (BBT-1-18011)
RELEVANCE OF STUDYING DISEASES RRELATED TO NERVOUS SYSTEM

The nervous system is the most crucial aspect of the human body. It not only works to produce
thoughts, emotions, and behavior, but also controls important body functions, like breathing.
Studying the nervous system advances understanding of our basic biology and body function.
Knowing how things typically work can help shed light on what may happen when there are
problems. It can help researchers find ways to prevent or treat problems that affect the brain,
nervous system, and body.
In addition to examining the normal development and activity of the nervous system,
neuroscience studies diseases, disorders, and injuries that affect parts of the nervous system,
how it develops, and how well it functions. There are more than 1,000 disorders of the brain
and nervous system, including:
• Intellectual and developmental disabilities, such as Down syndrome and Fragile X
syndrome
• Behavioral disorders, such as attention deficit/hyperactivity disorder and autism
spectrum disorders
• Learning disabilities and reading disorders
• Mental health problems, such as schizophrenia, obsessive-compulsive disorder, and
addiction
• Degenerative diseases, such as Alzheimer’s disease, Parkinson’s disease, and Niemann-
Pick disease
• Musculoskeletal disorders, such as muscular dystrophy and stroke
• Structural defects, such as neural tube defects, which include spina bifida,
hydrocephaly, and myelomeningocele
• Injuries, including traumatic brain injury and spinal cord injury, as well as how the
body processes pain
• Cancers, including brain tumors such as paragangliomas
• Immune system disorders, such as HIV/AIDS
• Epilepsy, seizures, and stroke
By studying these diseases we are able to have a better understanding of how to prevent and
treat these disorders and diseases and it is crucial for maintaining the overall health and well-
being of all people.
ALZHEIMER’S DISEASE

CASE STUDY:

Sean Warner is a 68 year old, retired structural engineer who has been experiencing problems
with his short term memory. He has been having problems remembering names and the location
of certain belongings which his family had dismissed and attributed to the ageing process. Mr.
Warner’s medical history indicates that he had cases of minor strokes between the age of 45
and 47 which required him to take 75 gm of aspirin and he has been on it every day ever since.
Ever since this incidence, he is otherwise in good health, and although advised to stop smoking
has not done so. His family history includes a maternal uncle with dementia who is in his late
90s.
Initially, Mr. Warner exhibited minor traits of forgetfulness. After a few months, he started
experiencing trouble with remembering names of the people he just met, losing many of his
belongings and having trouble getting through some of his general activities.
These incidences, even though evident were shaken off by Mr. Warner and his family as signs
of old age. During his first diagnosis, his medical history was researched. His diet, use of
medicines, daily habits were enquired about. Blood pressure, temperature, blood and urine
testing was done. The standard laboratory work up was negative except for a mildly high blood
pressure. The Mini-Mental State Exam (MMSE) score was 27/30 (generally considered as
normal). The initial diagnostics concluded that there were no clear signs of cognitive loss.

A year and a half later, Sean Warner got stranded on an unknown road while returning home
with groceries and returned back home late after spending some time recollecting where he
went wrong. After being asked about the situation he replied that while driving due to some
confusion, he got stranded on an unknown road and couldn’t recall his way home. After this
incidence and noticing his abnormal behavior, his family decided to run another test.
During the reassessment, he described that his condition has worsened thus, resulting in a few
incidences which affected his day to day activity. He stopped running the daily errands and his
wife observed him to be more nervous than usual. The neurological exam shows that the patient
now shows slight buccolingual and limb apraxia. A diagnosis of early Alzheimer’s disease is
made.
Eventually, he is diagnosed by an AD specialist. His symptoms now encompass some word-
finding difficulties and the need for supervision to look after financial matters and household
work. His affect is described as sad with the tendency of sleeping more during the daytime. A
psychogeriatric consultation ruled out depression. His MMSE is now 24/30(mild dementia).
After a variety of tests including MRI, neurological and physiological tests it is concluded that
Mr. Warner is exhibiting dementia due to Alzheimer’s disease. Being in the initial stages of
Alzheimer’s disease, he has been prescribed drugs accordingly.
He is prescribed galantamine 4mg twice a day for 4 weeks, increasing to 8mg twice a day for
at least 4 weeks; maintenance 8-12mg twice a day. Galantamine improves the function of nerve
cells in the brain. It works by preventing the breakdown of a chemical called acetylcholine.
People with dementia usually have lower levels of this chemical, which is important for the
processes of memory, thinking, and reasoning. Although galantamine helped with slight
improvement, the Treatment was stopped after one week, due to side-effects like severe skin
rashes.
A new alternative treatment was introduced to cope with his worsening condition. Mr. Warner
was presented with a prescription for memantine (initially 5mg once daily, increased in steps
of 5mg every week to a maximum dose of 20mg per day). Memantine works differently than
galantine. Glutamate is another chemical that helps to send messages between nerve cells in
the brain. However, when nerve cells are damaged by Alzheimer’s disease, too much glutamate
is produced. This causes more damage to the nerve cells. Memantine protects nerve cells by
blocking the effects of too much glutamate. These drugs have considerably improved his
condition.

Mr. Warner’s treatment is still progressing and his family has been opting for newly introduced
drugs to battle AD which will hopefully be able to stabilize his condition and reduce his
symptoms.

ALZHEIMER’S STUDY CASE QUESTIONS AND RELATED STUDY.

WHAT IS ALZHEIMER’S DISEASE?

Alzheimer’s disease is an irreversible, progressive brain disorder that slowly destroys memory
and thinking skills and eventually, the ability to carry out the simplest tasks. It is generally
classified in two categories:
• Late onset AD: symptoms first appear in their mid-60s.
• Early onset AD: symptoms first appear between a person’s 30s and mid-60s. It is very
rare.
 Dementia is a general term for a decline in mental ability severe enough to interfere with daily
life. Memory loss is an example. Alzheimer's is the most common type of dementia.

WHAT IS THE CAUSE FOR AD?

Scientists believe that for most people, Alzheimer's disease is caused by a combination of
genetic, lifestyle and environmental factors that affect the brain over time.
The exact causes of Alzheimer's disease aren't fully understood, but at its core are problems
with brain proteins that fail to function normally, disrupt the work of brain cells (neurons) and
unleash a series of toxic events. Neurons are damaged, lose connections to each other and
eventually die. The damage most often starts in the region of the brain that controls memory,
but the process begins years before the first symptoms. The loss of neurons spreads in a
somewhat predictable pattern to other regions of the brains. By the late stage of the disease, the
brain has shrunk significantly.
Researchers are focused on the role of two proteins

• Plaques. Beta-amyloid is a leftover • Tangles. Tau proteins play a part in a

fragment of a larger protein. When neuron's internal support and transport
these fragments cluster together, they system to carry nutrients and other
appear to have a toxic effect on essential materials. In Alzheimer's
neurons and to disrupt cell-to-cell disease, tau proteins change shape and
communication. These clusters form organize themselves into structures
larger deposits called amyloid called neurofibrillary tangles. The tangles
plaques, which also include other disrupt the transport system and are toxic
cellular debris. to cells.
WHAT TYPE OF TREATMENT SHOULD BE PROVIDED FOR AD?
Medical management can improve the quality of life for individuals living with Alzheimer’s
disease and their caregivers. There is currently no known cure for Alzheimer’s disease.
Treatment addresses several different areas:
• Helping people maintain mental function.
• Managing behavioral symptoms.
• Slowing or delaying the symptoms of the disease.
Patients with dementia who develop non-cognitive symptoms (such as delusions or anxiety) or
'behavior that challenges' (such as aggression or agitation) should only be offered a
pharmacological intervention if they are severely distressed or if there is an immediate risk of
harm to the patient or others. Less severe non-cognitive symptoms are treated with non-
pharmacological interventions, such as multisensory stimulation or aromatherapy.
Antipsychotic drugs are used to provide maximum relief to AD patients because they
constantly undergo anxiety attacks and episodes of aggression and depression. However
antipsychotic drugs do not help a great deal as they have a lot of side effects. Hence it is advised
against prescribing them early stages. Only during the late stages and high levels of distress is
it advised to start their dosage in small quantities.
PARKINSON’S DISEASE

Parkinson’s disease is the slow progressing chronic nervous disease, which occurs among the
elderly people. The disease is supposed to be one of the diseases of extrapyramidal system.
Parkinson’s disease is caused by the ruining and death of the neurons, producing
neurotransmitter dopamine in the branches of the nervous system. The insufficient production
of dopamine leads to the increased effect of basal ganglia on the human brains. The major
symptoms which are typical for Parkinson’s disease are muscular rigidity; hypokinesia; tremor
and moving disorder. The modern medicine can not cure the disease or reduce its progressing,
but there are remedies which can make the patient’s life easier and less troublesome.

PPATHOPHYSIOLOGY:

Destruction of dopaminergic neuronal cells in the substantia nigra in the basal ganglia

Degeneration of the dopaminergic nigrostriatal pathway

Depletion of dopamine store

Imbalance of excitatory(acetylcholine) and inhibiting(dopamine) neurotransmitters in the

corpus striatum

Impairment of extrapyramidal tracts controlling complex body movements

Tremors, rigidity, bradykinesia

CASE REPORT:

This case report discusses about Mr Parvindar kaur , he is 64 yrs old and is a businessman by
occupation . My patient presented with abnormal rhythmic movement of upper and lower limbs
since 1 year. It started on the distal part of both upper limbs simultaneously. It was noticed
more during rest that got aggravated during movements. Recently the lower limbs were also
affected and was noted more on both great toes. Patient also gives history of difficulty in
walking in the form of difficulty in initiation and once he starts walking he stoop forward with
small and fast steps. However, he freezes during turning and has tendency to fall forward
(propulsion) and backward (retropulsion).
DIAGNOSIS:

REPORT OF PATIENT:
MEDICAL MANAGEMENT:

My patient has been prescribed following medications

1. Tab Syndopa plus 1 tab 5 times a day

2. Tab Pramipexole 0.5 mg 2 times a day

3. Tab Domperidone 10 mg twice a week

4. Syrup lactulose 2 tsf/ twice a day for 2 months

5. 2% Xylocaine ointment

Other than medications my patient has been suggested walking and maintain upright position
during sitting, meditation and movement exercises.

DISCUSSION:

The progression of Parkinson's disease and the degree of impairment vary from person to
person. Many people with Parkinson's disease live long productive lives, whereas others
become disabled much more quickly. Complications of Parkinson’s such as falling-related
injuries or pneumonia. However, studies of patent populations with and without Parkinson’s
Disease suggest the life expectancy for people with the disease is about the same as the general
population.

Most people who develop Parkinson's disease are 60 years of age or older. Since overall life
expectancy is rising, the number of individuals with Parkinson's disease will increase in the
future. Adult-onset Parkinson's disease is most common, but early-onset Parkinson's disease
(onset between 21-40 years), and juvenile-onset Parkinson's disease (onset before age 21) can
occur.

Although Parkinson's disease progresses slowly, it will eventually affect every aspect of life -
from social engagements, work, to basic routines. Accepting the gradual loss of independence
can be difficult. Being well informed about the disease can reduce anxiety about what lies
ahead. Many support groups offer valuable information for individuals with Parkinson's disease
and their families on how to cope with the disorder. Local groups can provide emotional
support as well as advice on where to find experienced doctors, therapists, and related
information. It is also very important to stay in close contact with health care professionals to
monitor the progression of the disease and to adjust therapies to maintain the highest quality of
living.

Although Parkinson's disease progresses slowly, it will eventually affect every aspect of life -
from social engagements, work, to basic routines. Accepting the gradual loss of independence
can be difficult. Being well informed about the disease can reduce anxiety about what lies
ahead. Many support groups offer valuable information for individuals with Parkinson's disease
and their families on how to cope with the disorder. Local groups can provide emotional
support as well as advice on where to find experienced doctors, therapists, and related
information. It is also very important to stay in close contact with health care professionals to
monitor the progression of the disease and to adjust therapies to maintain the highest quality of
living.
EPILEPSY

Epilepsy is a central nervous system (neurological) disorder in which brain activity

becomes abnormal, causing seizures or periods of unusual behaviour, sensations, and
sometimes loss of awareness.

Anyone can develop epilepsy. Epilepsy affects both males and females of all races, ethnic
backgrounds and ages.

Seizure symptoms can vary widely. Some people with epilepsy simply stare blankly for a
few seconds during a seizure, while others repeatedly twitch their arms or legs. Having a
single seizure doesn't mean you have epilepsy. At least two unprovoked seizures are
generally required for an epilepsy diagnosis.

Treatment with medications or sometimes surgery can control seizures for the majority of
people with epilepsy. Some people require lifelong treatment to control seizures, but for
others, the seizures eventually go away. Some children with epilepsy may outgrow the
condition with age.

CASE STUDY:

Patient R is a woman, 38 years of age, with a history of seizures since birth. She is otherwise
in good health. Her current diagnosis is temporal lobe epilepsy. Patient R experiences focal
seizures ("complex partial" and focal seizures that evolve bilaterally). She states that she is
aware that a seizure is going to occur because she has a very brief "strange sensation." This
sensation is her aura. After the aura, the patient cannot recall any other events until the postictal
period. During the postictal time, Patient R is fatigued and confused and often experiences
headaches.

Witnesses have noticed a typical pattern to Patient R's seizures. First, she becomes very quiet
and blank. She will not communicate or respond to other individuals. She experiences
vocalizations and yells in a very loud voice, "Jesus, help me." During the vocalizations, Patient
R will experience automatisms that include pulling at her clothing. Usually, the seizure ends
after approximately 60 seconds, and she regains consciousness within several minutes. At other
times, the seizure will evolve, and she will experience tonic posturing followed by clonic
movements. The tonic-clonic phase is quite severe and may last several minutes. The postictal
period after the evolved seizure is prolonged and may last for several hours. Patient R
experiences disorientation, confusion, and somnolence. She has experienced status epilepticus
twice in the past.

At times, Patient R's seizures occur almost every day. However, some days will be seizure free,
and she may have several days at a time with no seizure events. Unfortunately, this does not
occur consistently. Most days she will experience at least one seizure, and often she has several
during the same day.

She has been on a number of AEDs (automatic external defibrillator) in the past, including
phenytoin, phenobarbital, valproic acid, and experimental medications. Her current
medications include carbamazepine and topiramate, with lorazepam as needed. She also takes
an over-the-counter multivitamin and, occasionally, acetaminophen for headache. Her sister
has been instructed on administering lorazepam when Patient R is experiencing several
consecutive seizures that occur over a 15-minute period or after her second bilaterally evolved
focal seizure for the day. Her sister administers the lorazepam approximately once every two
weeks. However, Patient R's seizures are quite variable with no known pattern. One week, she
may not require any lorazepam; the next week, she may require it several times.

Patient R is very compliant with her medications. She uses a pillbox and can correctly describe
her daily medications and doses. She has used a seizure calendar method of tracking her
seizures for years. Each day she records the number of seizures she has and describes them.
Patient R has no family history of epilepsy, and she has not had any surgical treatments.

During Patient R's physical examination, her physician discovers that the patient has difficulty
with coordination. There are deficits in cognitive processes, such as calculation and abstract
thinking. The exam is otherwise unremarkable.

Patient R has a twin sister who is neurologically normal. The twin sister provides a large
amount of social and emotional support and is always present to accompany the patient at her
visits. Patient R is a very independent woman who lives in an assisted living center and wears
a protective helmet. She has completed the 5th grade. She does not use nicotine products, drink
alcohol, or use illicit substances. She has experienced difficult situations in the past related to
a divorce and loss of her only child.
The patient has been involved in the trial of several new experimental AEDs with varying
success. The latest study she participated in had a positive effect on her seizure frequency.
Unfortunately, the pharmaceutical company discontinued the medication from the study. As a
result, other alternatives were sought. Her current healthcare providers evaluated Patient R and
placed her in the epilepsy monitoring unit for simultaneous EEG/video monitoring.

In the epilepsy monitoring unit, Patient R's AEDs are slowly withdrawn. She begins to
experience her typical events, including vocalizations. During these events, a large amount of
motor activity is exhibited, and EEG readings are difficult to ascertain. The medical team
suspects a diagnosis of pseudo seizures. The patient completes the neuropsychologic testing,
and the evaluation continues for several days. The patient experiences a focal seizure that
evolves to a bilateral convulsive seizure, and the EEG readings clearly reveal epileptogenic
changes. Sphenoidal electrodes are placed to obtain localizing information. Patient R continues
to have a large number of seizures and requires frequent administration of intravenous
benzodiazepines to maintain seizure control.

After more than a week of monitoring, a seizure focus is determined. Unfortunately, multiple
focal areas exist in the bilateral temporal and frontal lobes, eliminating the surgical option. The
patient is restarted on her AEDs and stabilized, and alternative treatments are discussed. The
patient and healthcare team agree that a vagus nerve stimulator would be a positive option.
Alterations in the medication regimen are discussed. The patient and sister are offered the
option to attempt dosing with felbamate with close monitoring. Patient R does not want to
attempt felbamate and opts for slowly removing the topiramate and attempting a trial with
lamotrigine while awaiting the vagus nerve stimulator placement. This medication is somewhat
helpful, but the vagus nerve stimulator provides substantial relief.

MULTIPLE SCLEROSIS

CASE STUDY:

Multiple Sclerosis (MS) is an autoimmune disorder characterized by demyelination and

subsequent axonal injury and loss in the central nervous system [1]. This injury to the central
nervous system causes white matter lesions, also known as plaques, which are responsible for
the patients’ symptoms. There are a few different classifications of MS based on occurrence of
relapses or progression. The most common form is Relapsing Remitting Multiple Sclerosis
(RRMS) whereby the patient will have new symptoms emerge or a worsening of past
symptoms, followed by full, or near full recovery [1]. The majority of RRMS cases eventually
transition to a progressive form called Secondary Progressive Multiple Sclerosis (SPMS) [1].
About 15% of MS cases are Primary Progressive (PPMS) where function continues to decline
from onset [1].
This case study discusses a 27 year-old female diagnosed with Relapsing Remitting Multiple
Sclerosis two years ago.
Patient first noticed symptoms that persisted for 24 hours including vision loss in one eye
accompanied by facial weakness, numbness and difficulty with speech (dysarthria) in April of
2017. After the symptoms did not subside, the patient reached out to her family doctor who
then suggested further testing. The patient underwent magnetic resonance imaging (MRI) and
a lesion was revealed on the brainstem. These results lead to the patient being classified as
having Clinically Isolated Syndrome [2]. Patients such as this one being discussed are classified
as having Clinically Isolated Syndrome when an initial event leads to a clinical presentation of
symptoms and the MRI reveals lesions [2]. It wasn’t until three months after the initial MRI
that the patient started experiencing extreme fatigue and balance problems possibly due to
lower extremity weakness. The patient underwent a second MRI which revealed another lesion
in her right cerebral hemisphere. Due to a relapse in symptoms, the patient was formally
diagnosed with Relapsing Remitting Multiple Sclerosis (RRMS) [3]. It has been two years
since the patient’s diagnosis and she has opted to come to physiotherapy to help control her
fatigue, lower limb weakness, coordination and maintain general fitness.
The purpose of this case study is to present the patients symptoms of MS (fatigue, balance and
lower limb weakness) and their response to physical therapy treatment. Similar cases of
moderate to severe MS presenting with fatigue and weakness have shown significant
improvements through physical therapy treatment [4]. In general, both strength and endurance
training result in fatigue reduction; however, evidence is insufficient in specifying which
training modality has the strongest effect [4]. Although there is a need for specific balance
exercises in MS patients, there is evidence that suggests progressive aerobic and resistance
exercises have positive effects on balance in patients who present with mild to moderate
symptoms. [5]
CLIENT CHARACTERERISTICS:

Patient is a 27-year-old Caucasian female diagnosed with Relapsing Remitting Multiple

Sclerosis that began two years ago. The patient is a current smoker and has a history of
depression. The patient self-referred herself to physiotherapy to help manage her weakness,
coordination and fatigue. Additionally, the patient is looking to learn how to self-manage her
symptoms.

Figure 1. Patient completed LEFS with a total score of 43/80[6].

Figure 2. Patient completed PHQ-9 with a total score of 10[7].
Figure 3. Fatigue Severity Scale (FSS): Mean score of 5.3 (>/=4 classified as substantial
fatigue) [8] [9].

DIAGNOSTIC TESTS:

• Magnetic Resonance Imaging (MRI)

• Lumbar puncture (spinal tap)
CLINICAL IMPRESSION:
Physiotherapy Diagnosis:
Patient was a previously active 27 year-old female diagnosed with Relapsing Remitting
Multiple Sclerosis two years ago when she first started showing signs of the disease. Three
months within that year, the patient experienced her first relapse of the disease which ultimately
lead to chronic fatigue, weakness and impaired coordination of the lower extremities affecting
functional mobility and impacting her ability to independently engage in activities of daily
living.
PROBLEM LIST:
• Fatigue impacting overall quality of life
• Lower limb weakness impairing functional mobility
• Impaired coordination affecting activities of daily living
• Current smoke (1 pack every 2 days)
OUTCOMES:
12 Week Re-Assessment
1) The patient has been partaking in Tai Chi classes for 30 minutes per week.
• Patients PHQ-9 score decreased from 10 to 8.
• Coordination was re-evaluated using finger to nose test and heel knee shin test which
. both showed improvements, however, the improvements are not yet clinically significant.
• BERG score has improved to 50/56 indicating 24-32% increase in fall risk.
2) After 12 weeks of combined strength and aerobic training twice a week, the patient has
noticed an improvement in her fatigue. This was confirmed by retaking the Fatigue Severity
Scale where she obtained an average score of 4.5 compared to her original score of 5.3 12
weeks prior.
3) Additionally, there was no detectable change in the manual muscle testing results, however,
patient was able to perform 8 consecutive moderate depth, unsupported squats.
The patient is encouraged to maintain consistency throughout treatment. It is suggested that
patient increases their Tai Chi to 45 minutes per week and continues to increase walking time
on graded walking program to continue making improvements.

DISCUSSION:
This case study examines a young female patient with a typical presentation of Relapsing
Remitting Multiple Sclerosis. The patient sought treatment from physiotherapy after her most
recent attack left her with symptoms of fatigue, lower extremity weakness, loss of coordination
and balance. Through objective measures, the physiotherapists working with this patient were
able to create goals based on their findings and ultimately create a treatment plan. Fatigue
associated with MS can be one of the most debilitating symptoms and can affect a patient's
quality of life tremendously. It is important to address this in treatment through the use of
aerobic and resistance training as well as with Thai Chi. In addition, interventions such as these
pose secondary positive effects on coordination, balance and depression[15] . Due to MS being
a progressive disease, it is important to educate the patient on both self management techniques
such as energy conservation strategies and the importance of keeping their core temperature
low to avoid worsening of the disease [16]. Furthermore, knowing you have a progressive
disease can be mentally taxing. Therefore, it is vital to incorporate SCT with an emphasis on
goal setting, outcome expectations and self efficacy [17].
The implications of this case suggest multiple modes of exercise training can have a significant
impact on quality of life, fatigue and strength in patients with mild to moderate Relapsing
Remitting Multiple Sclerosis [4]. Currently, there is a plethora of research on mild to moderate
severity Multiple Sclerosis, however, the literature is lacking for severe cases. Thus, future
inquiry should address intervention-based research in severe Multiple Sclerosis.
JAPANESE ENCEPHALITIS

Japanese encephalitis (JE) is one of the leading causes of acute encephalopathy in the tropics.
It mainly affects children <15 years and is mostly asymptomatic. 1 When symptomatic, it
usually resolves within weeks and ventilator support is not commonly required. Hospitalization
is mainly due to neurological symptoms and respiratory involvement may or may not occur.
After a prolonged ICU stay weaning from ventilator can be difficult and can have multiple
causes. Critical illness polyneuropathy is the most common peripheral neuromuscular disorders
encountered during difficult weaning in ICU setting.
CASE REPORT:

A 17-year-old boy presented with generalised headache, evening rise of temperature, altered
sensorium and un-coordination of movement for 5 days. He later developed difficulty in
breathing for which he was referred to our centre.
On admission, he was febrile with a temperature of 101°F, pulse of 134/min blood pressure of
88/47. On pulse oximetry 91% saturation with signs of respiratory distress. The peripheries
were cool, pale and clammy. Arterial blood gas analysis revealed PO2– 58.7, PCO2-59.7,
oxygen saturation of 88%. Tracheal intubation was performed to aid mechanical ventilation;
SIMV mode with PS-18, PEEP of 5 cmH2O and FiO2 of 80% and RR-14.
Systemic inflammatory response syndrome was provisionally diagnosed and treatment was
initiated as recommended by the surviving sepsis guidelines. A central venous catheter (CVC)
was inserted via right internal jugular vein and a MAP of >70 mmHg was achieved with the
help of fluid resuscitation and norepinephrine infusion. The patient was started on empirical
antimicrobials which were later modified according to culture sensitivity.
On neurological examination his Glasgow Coma Scale (GCS) was E3VtM2 with rigid limbs.
There were no signs of meningism. The rest of the systemic examination was within normal
limit.
His baseline routine investigations were;
On day 2, MRI showed patchy T2 and Flair hyperintense involving basal ganglia and thalami
with patchy restriction on DWL specific of JE. CSF examination showed features 0f viral
encephalitis and a fourfold increase in IgM antibodies against JE. Antibodies against dengue,
chikungunya virus (CHIKV), hepatitis virus and herpes simplex were negative, confirming the
diagnosis of acute Japanese encephalitis with sepsis.
After 2 weeks there was improvement in his GCS, vital parameters, hemodynamic status and
lab values indicating sepsis control; weaning was considered and the patient was given
spontaneous breath trial. His ill-sustained efforts showed no improvement in saturation and
ABG’s. The patient underwent tracheostomy on day 15 after failing multiple attempts at
spontaneous breathing trials. He was continued on SIMV mode of ventilation for another 3
weeks and his nutrition was stepped up keeping in view his hypercatabolic state and increased
demand due to increase in work of breathing.
Over a period of next 20 days patient was weaned off to spontaneous mode of ventilation;
gradually decreasing the pressure support from 20 cmH2O to 8 cmH2O and increasing his
trigger sensitivity. Improvement was evident in vital parameters, oxygen saturation, ABG’s
and chest x-ray. During this weaning period he was put off the sedation, whenever he developed
a temperature of 38.5°C or more, systolic blood pressure greater than 140 mmHg, pulse rate of
at least 130 beats per minute, respiratory rate of at least 20 breaths per minute, agitation,
diaphoresis, and dystonia. These symptoms continued for more than three times a day pointing
to clinical diagnosis of sympathetic storm after traumatic brain injury. Serum levels of
epinephrine during episodes were not done but patient responded well to clonidine 0.1 mg. T-
piece trial was considered after patient responded to clonidine and his hemodynamic became
stable. It was initially given for 10-15 sec per day. Later over a period of 50 days the trail
duration was increased up to 24 hours maintaining saturation and adequate P/F ratio in ABG’s.
During his weaning period routine ICU care, chest and limb physiotherapy, and adequate
nutritional support continued throughout. Anabolic steroids were given every 21 days to build
his muscle mass and aid his weaning.

DISCUSSION:
Japanese encephalitis (JE) virus has been known to be the leading cause of viral neurologic
disease and disability in Asian countries. More than 100 days of mechanical ventilation in a
patient of acute encephalitis makes it a case of prolonged weaning. The causes can be multiple
in this patient and common causes in context would be discussed. Reduced central drive due
to unresolved encephalopathy may be present. Patients do not exhibit any ventilatory activity
upon discontinuation from the ventilator, and this persists despite hypercapnia and
hypoxaemia. However, Japanese encephalitis usually resolves in days to weeks and residual
neurological disease could be present. Improvement in GCS and no development of any other
neurological signs makes this diagnosis unlikely. Reduced drive can be due to metabolic causes
like alkalosis and electrolyte disturbances. Hypophosphatemia, hypomagnesaemia and
hypokalaemia all can cause muscle weakness. 3 Hypothyroidism and hypoadrenalism may also
contribute to difficulty in weaning. Recent ABG and electrolyte were well within normal limit
for this patient. Malnutrition causes reduction of muscle mass, endurance, and muscle strength.
It also causes decreased immunity, predisposing the patient to further infections. Nutrition
repletion in critically ill patients showed improved respiratory forces and facilitated weaning.
Our patient was started on enteral nutrition from day 5 with anti-oxidant and trace element
supplementation in accordance to his needs for a hypercatabolic state and sepsis. Myopathies
can be steroid or Neuromuscular myopathy and critical illness myopathy. Steroid induced
myopathy in icu setting have been associated with long term use of steroid and neuromuscular
agents along with aminoglycosides. 5 However, 5 days use of 100 mg hydrocortisone and
normal levels of creatinine phosphokinase (CPK), AST, ALT ruled out steroid induced
myopathy. Critical illness polyneuropathy are the most common peripheral neuromuscular
disorders encountered in the ICU setting and usually involve both muscle and nerve3. Risk
factors can be severity of illness, multiple organ dysfunction, SIRS, exposure to corticosteroids,
presence of hyperglycaemia and prolonged ICU stay. 6,7 The signs and symptoms are distal
muscle weakness, reduced or absent deep tendon relaxes, weakness of respiratory muscle
leading to difficulty in weaning.8,9 It is an acute axonal sensory motor polyneuropathy so
electroneurography is the gold diagnostic standard10 and neurologic examination is neither
sensitive nor specific.11 To confirm the diagnosis and differentiate it from other illnesses,
electromyography (EMG) and nerve conduction studies were undertaken in this patient, which
showed reduction of amplitude and duration of muscle compound action potentials and ±
decreased amplitude of sensory nerve action potentials which is common in both critical illness
myopathy and critical illness polyneuropathy. CPK levels were relatively normal (140 units/L)
pointing more towards neuropathy. Fatigue of patients undergoing weaning from MV is a
major factor in failure to wean. Ventilator associated diaphragm dysfunction may be one of the
causes. 18 to 69 hours of complete diaphragmatic inactivity and mechanical ventilation results
in marked atrophy of human diaphragm myofibers.12 These findings are consistent with
increased diaphragmatic proteolysis during inactivity. Oxidative stress due to critical illness
can also be contributing, however, tocopherol, vitamin and trace element supplementation have
shown a role in reducing these risks.13-15
Difficult weaning as seen in this patient can be due to multiple causes and attributing to a single
cause is difficult. However, as evidenced by the NCV critical illness polyneuropathy can be
the main cause. Few of the risk factors were present in this case. Treatment is nonspecific and
includes supportive care, personal hygiene, limb physiotherapy. Early mobilization of these
patients may lead to a better outcome.