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ClinicalPractice
Clin. Pract. (2020) 17(2), 1463-1465 ISSN 2044-9038
1463
CASE REPORT
Parkinsonism-hyperpyrexia
syndrome-A rare case report
Aishwarya Ghule,
Sourya Acharya*,
Vidya Hulko and
Yash Gupte
Department of Medicine, Daa Meghe
Instute of Medical Sciences (Deemed
to be University) Jawaharlal Nehru
Medical College Sawangi, India
*Author for correspondence:
souryaacharya74@gmail.com
Introduction
Parkinsonism-Hyperpyrexia Syndrome (PHS)
was rst described in 1981. If not suspected
early it can be life-threatening [1]. Sometimes
it is misdiagnosed as a neuroleptic malignant
syndrome (NMS), sepsis with multi-organ
failure, infection. It is a hypodopaminergic
state that results due to the sudden stoppage of
levodopa [2]. Clinical manifestations of PHS are
very akin to neuroleptic malignant syndrome
(NMS) [2,3]. PHS presents with myriads of
features ranging from hyperpyrexia, rigidity,
altered sensorium, autonomic dysfunction and
elevated creatine kinase (CK). Early diagnosis
and prompt reinstitution of antiparkinsonian
medications are the cornerstones of the
treatment of this condition [2].
Case Report
A 66-year-old male presented to us with a
history of high-grade fever and altered sensorium
for 12 hours. ere was no history of headache,
vomiting, diplopia or seizures. ere was no
history of diabetes, hypertension. He was a
known case of PD for 12 years and was taking
Levodopa (200 mg)+Carbidopa (50 mg) twice a
day. He had last visited his treating physician 2
months back before the presentation.
ree days back he went to a relative’s place
and had forgotten to pack his medications. He
had come back to his home after 3 days and
presented with the sudden development of
symptoms in the form of irrelevant talking, not
recognizing relatives and fever for which he was
brought to this hospital.
On examination, the Glasgow Coma Scale
was 11(E-3/M-5/V-3). Temperature -104.2°F,
Pulse -166 beats/min, regular. Blood pressure
was 100/70 mmHg. ere was no signicant
lymphadenopathy, JVP was normal and there
was no oedema foot. ere were no rashes
over the body. CVS, RS, and P/A examination
were normal. Neurologic examination revealed
hypertonia and cogwheel rigidity in all 4 limbs.
Plantars were bilateral exor. e patient did
not co-operate for the elicitation of apping
tremors.
On investigations complete blood count
was normal. LFT and serum electrolytes were
normal. Blood urea was 78 mg/dl and serum
creatinine was 3.9 mg/dl. X-ray chest and USG
abdomen did not reveal any abnormality. Total
CPK was 2800 U/L (normal -39-308 U/L). CT
brain revealed cortical atrophy. Malaria antigen
test, IgM dengue was negative. ABG revealed
metabolic acidosis.
Abstract
Parkinsonism-Hyperpyrexia Syndrome (PHS) though rare, is a catastrophic treatment-related complication of Parkinson’s
Disease (PD). It is a hypodopaminergic state which occurs due to the abrupt withdrawal of dopaminergic medications
in patients with PD. Clinically PHS resembles neuroleptic malignant syndrome (NMS). We report a case of a 66-year-old
male who was diagnosed with PD 12 years earlier and was on Levodopa (200 mg)+Carbidopa (50 mg) twice a day and
Tablet Selegeline 5 mg once in the morning. He had abruptly stopped taking medications because he went on a trip to
his relative’s place and presented to us with hyperpyrexia and acute kidney injury. He recovered after the reinstitution of
levodopa.
Keywords: parkinsonism, hyperpyrexia, hypodopaminergic state, acute kidney injury
10.4172/clinical-practice.1000416 Clin. Pract. (2020) 17(2)1464
CASE REPORT Ghule, Acharya, Hulkoti & Gupte
He was treated with antipyretics, IV uids,
empirical antibiotics, external cooling with
ice packs, injection soda bicarbonate and two
sessions of hemodialysis. In the next 24 hours
his fever did not come down and mental
obtundation persisted. At this point, the history
was re-evaluated and levodopa was reinstituted.
Over the next 72 hours his fever subsided, the
mental condition improved, creatinine came
down to 1.9 mg/dl and total CPK was decreased
to 600 U/L. rough the next 5 days, his
recovery was signicant and he was discharged
with normal sensorium, normal temperature,
and normal biochemical prole.
Discussion
PHS is a medical emergency that resembles
NMS. Diagnosis is usually straight forward if
the prior history of PD is known and the history
of the sudden stoppage of levodopa is elicitated.
Our patient had hyperpyrexia, rigidity, altered
sensorium, rhabdomyolysis induced AKI
with high CPK levels which required holistic
management including dialysis. But dramatic
recovery occurred only after the reinstitution
of levodopa. Literature suggests if the diagnosis
of PHS is delayed, multiple complications like
AKI, pulmonary embolism, and DIC can occur,
leading to death [2].
e most common cause of PHS is sudden
withdrawal or sudden decrease in the doses
of levodopa or dopa agonists. Amantadine
an antiviral drug used to treat PD also can
predispose to PHS after sudden withdrawal.
Untreated it is potentially fatal [3].
PHS is also known as Neuroleptic Malignant
Like Syndrome (NMLS) [1] because it
resembles the clinical presentation of NMS.
Diagnosis is straightforward if a careful drug
history is elicitated regarding discontinuation
of antiparkinsonian medication [4,5]. Our
case had features suggestive of NMS and the
reinstitution of levodopa improved the clinical
picture. If the diagnosis of PHS is delayed then
catastrophic complications in the form of AKI,
aspiration pneumonitis and DIC may prove to
be fatal [6,7].
Medical literature has documented evidence
of the occurrence of PHS mostly in the form of
case reports and case series [8-15].
e most common etiology of PHS in
these cases was abrupt withdrawal/reduction
and/or alteration of anti-parkinsonian
medication especially levodopa /dopa agonists
[4,5]. Two case reports of PHS also describe the
failure of deep brain stimulator due to exertion
of its battery or withdrawal of DBS. All these
cases improved after the reinstitution of therapy,
though some cases reported mortality [16-22].
e underlying pathophysiology of PHS
is a sudden hypodopaminergic state which
occurs by the abrupt cessation of anti-
parkinsonian therapy. Muscular rigidity of
PHS is due to decreased dopaminergic activity
and hyperthermia is due to dysregulation of
preoptic, anterior hypothalamic and posterior
hypothalamic functions. e aection of
mesolimbic and mesocortical pathways through
dopamine deletion explains the mental status
changes [16,17].
Conclusion
It is important to dierentiate PHS from
NMS. A careful clinical examination and a drug
history resolve the issue in most of the cases. Any
patient of Parkinson’s disease who presents with
a clinical picture of NMS should be entertained
as a high index of suspicion for PHS. Supportive
measures and reinstitution of anti-parkinsonian
drugs are the cornerstone of therapy.
10.4172/clinical-practice.1000416Clin. Pract. (2020) 17(2)
RESEARCH ARTICLE
Parkinsonism-hyperpyrexia syndrome-A rare case report
1465
CASE REPORT
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