Parkinsonism-Hyperpyrexia Syndrome-A Rare Case Report RESEARCHCASE ARTICLE REPORT

CASE REPORT

Parkinsonism-hyperpyrexia ClinicalPractice syndrome-A rare case report

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

Introduction Three days back he went to a relative’s place Aishwarya Ghule, and had forgotten to pack his medications. He Sourya Acharya*, Parkinsonism-Hyperpyrexia Syndrome (PHS) had come back to his home after 3 days and Vidya Hulkoti and was first described in 1981. If not suspected presented with the sudden development of Yash Gupte early it can be life-threatening [1]. Sometimes symptoms in the form of irrelevant talking, not it is misdiagnosed as a neuroleptic malignant Department of Medicine, Datta Meghe recognizing relatives and fever for which he was Institute of Medical Sciences (Deemed syndrome (NMS), sepsis with multi-organ brought to this hospital. to be University) Jawaharlal Nehru failure, infection. It is a hypodopaminergic Medical College Sawangi, India state that results due to the sudden stoppage of On examination, the Glasgow Coma Scale was 11(E-3/M-5/V-3). Temperature -104.2°F, *Author for correspondence: levodopa [2]. Clinical manifestations of PHS are [email protected] very akin to neuroleptic malignant syndrome Pulse -166 beats/min, regular. Blood pressure (NMS) [2,3]. PHS presents with myriads of was 100/70 mmHg. There was no significant features ranging from hyperpyrexia, rigidity, lymphadenopathy, JVP was normal and there altered sensorium, autonomic dysfunction and was no oedema foot. There were no rashes elevated creatine kinase (CK). Early diagnosis over the body. CVS, RS, and P/A examination and prompt reinstitution of antiparkinsonian were normal. Neurologic examination revealed medications are the cornerstones of the hypertonia and cogwheel rigidity in all 4 limbs. treatment of this condition [2]. Plantars were bilateral flexor. The patient did not co-operate for the elicitation of flapping Case Report tremors. On investigations complete blood count A 66-year-old male presented to us with a was normal. LFT and serum electrolytes were history of high-grade fever and altered sensorium normal. Blood urea was 78 mg/dl and serum for 12 hours. There was no history of headache, creatinine was 3.9 mg/dl. X-ray chest and USG vomiting, diplopia or seizures. There was no abdomen did not reveal any abnormality. Total history of diabetes, hypertension. He was a CPK was 2800 U/L (normal -39-308 U/L). CT known case of PD for 12 years and was taking brain revealed cortical atrophy. Malaria antigen Levodopa (200 mg)+Carbidopa (50 mg) twice a test, IgM dengue was negative. ABG revealed day. He had last visited his treating physician 2 metabolic acidosis. months back before the presentation.

1463 Clin. Pract. (2020) 17(2), 1463-1465 ISSN 2044-9038 CASE REPORT Ghule, Acharya, Hulkoti & Gupte

He was treated with antipyretics, IV fluids, of antiparkinsonian medication [4,5]. Our empirical antibiotics, external cooling with case had features suggestive of NMS and the ice packs, injection soda bicarbonate and two reinstitution of levodopa improved the clinical sessions of hemodialysis. In the next 24 hours picture. If the diagnosis of PHS is delayed then his fever did not come down and mental catastrophic complications in the form of AKI, obtundation persisted. At this point, the history aspiration pneumonitis and DIC may prove to was re-evaluated and levodopa was reinstituted. be fatal [6,7]. Over the next 72 hours his fever subsided, the Medical literature has documented evidence mental condition improved, creatinine came of the occurrence of PHS mostly in the form of down to 1.9 mg/dl and total CPK was decreased case reports and case series [8-15]. to 600 U/L. Through the next 5 days, his recovery was significant and he was discharged The most common etiology of PHS in with normal sensorium, normal temperature, these cases was abrupt withdrawal/reduction and normal biochemical profile. and/or alteration of anti-parkinsonian medication especially levodopa /dopa agonists Discussion [4,5]. Two case reports of PHS also describe the failure of deep brain stimulator due to exertion PHS is a medical emergency that resembles of its battery or withdrawal of DBS. All these NMS. Diagnosis is usually straight forward if cases improved after the reinstitution of therapy, the prior history of PD is known and the history though some cases reported mortality [16-22]. of the sudden stoppage of levodopa is elicitated. Our patient had hyperpyrexia, rigidity, altered The underlying pathophysiology of PHS sensorium, rhabdomyolysis induced AKI is a sudden hypodopaminergic state which with high CPK levels which required holistic occurs by the abrupt cessation of anti- management including dialysis. But dramatic parkinsonian therapy. Muscular rigidity of recovery occurred only after the reinstitution PHS is due to decreased dopaminergic activity of levodopa. Literature suggests if the diagnosis and hyperthermia is due to dysregulation of of PHS is delayed, multiple complications like preoptic, anterior hypothalamic and posterior AKI, pulmonary embolism, and DIC can occur, hypothalamic functions. The affection of leading to death [2]. mesolimbic and mesocortical pathways through dopamine deletion explains the mental status The most common cause of PHS is sudden changes [16,17]. withdrawal or sudden decrease in the doses of levodopa or dopa agonists. Amantadine Conclusion an antiviral drug used to treat PD also can predispose to PHS after sudden withdrawal. It is important to differentiate PHS from Untreated it is potentially fatal [3]. NMS. A careful clinical examination and a drug history resolve the issue in most of the cases. Any PHS is also known as Neuroleptic Malignant patient of Parkinson’s disease who presents with Like Syndrome (NMLS) [1] because it a clinical picture of NMS should be entertained resembles the clinical presentation of NMS. as a high index of suspicion for PHS. Supportive Diagnosis is straightforward if a careful drug measures and reinstitution of anti-parkinsonian history is elicitated regarding discontinuation drugs are the cornerstone of therapy.

1464 10.4172/clinical-practice.1000416 Clin. Pract. (2020) 17(2) Parkinsonism-hyperpyrexia syndrome-A rare case report RESEARCHCASE ARTICLE REPORT

References Deep brain stimulation surgery com- Needham M. Parkinsonism-hyperpy- plicated by Parkinson hyperpyrexia rexia syndrome: The role of electrocon- Toru M, Matsuda O, Makiguchi K, syndrome. Neurol India. 59, 911-912 vulsive therapy. J Clin Neurosci. 13, 857- Sugano K. Neuroleptic malignant syn- (2011). 859 (2006). drome-like state following a withdrawal of antiparkinsonian drugs. J Nerv Ment Cheung YF, Hui CH, Chan JH. Par- Liu CJ, Crnkovic A, Dalfino J, Singh Dis. 169, 324-327 (1981). kinsonism-hyperpyrexia syndrome due LY. Whether to proceed with deep brain to the abrupt withdrawal of amanta- stimulator battery change in a patient Newman EJ, Grosset DG, Kennedy dine. Hong Kong Med J. 17, 167-168 with signs of potential sepsis and Par- PG. The parkinsonism-hyperpyrexia (2011). kinson hyperpyrexia syndrome: A Case syndrome. Neurocrit Care. 10, 136-140 report. AA Case Rep. 8, 187-191 (2017). (2009). Gil-Navarro S, Grandas F. Dyskine- sia-hyperpyrexia syndrome: Another Artusi CA, Merola A, Espay AJ, et al. Factor SA. Fatal Parkinsonism-hyper- Parkinson’s disease emergency. Mov Dis- Parkinsonism-hyperpyrexia syndrome pyrexia syndrome in a Parkinson’s disord. 25, 2691-2692 (2010). and deep brain stimulation. J Neurol. ease patient while actively treated with 262, 2780-2782 (2015). deep brain stimulation. Mov Disord. 22, Sechi GP, Tanda F, Mutani R. Fatal 148-149 (2007). hyperpyrexia after withdrawal of levodo- Govindappa ST, Abbas MM, Ho- pa. Neurology. 34, 249-251 (1984). surkar G, Varma RG, Muthane UB. Mohammed NB, Sannegowda RB, Parkinsonism hyperpyrexia syndrome Gibb WR, Griffith DN. Levodopa Hejamadi MI, Sharma B, Dubey P. Sin- following deep brain stimulation. Par- withdrawal syndrome identical to neu- gle-dose does matter! An interesting case kinsonism Relat Disord. 21, 1284-1285 roleptic malignant syndrome. Postgrad of Parkinson’s hyperpyrexia syndrome. J (2015). Neurol Disord. 2, 191 (2014). Med J. 62, 59-60 (1986). Wei L, Chen Y. Neuroleptic malig- Man SP. An uncommon adverse ef- Kim JH, Kwon TH, Koh SB, Park JY. nant-like syndrome with a slight eleva- fect of levodopa withdrawal in a patient Parkinsonism-hyperpyrexia syndrome tion of creatine-kinase levels and respira- taking antipsychotic medication: Neu- after deep brain stimulation surgery: tory failure in a patient with Parkinson’s roleptic malignant-like syndrome. Hong Case report. Neurosurgery. 66, 1029 disease. Patient Prefer Adherence. 8, 271- Kong Med J. 17, 74-76 (2011). (2010). 273 (2014). Factor SA. Fatal parkinsonism-hyper- Friedman JH, Feinberg SS, Feldman Arora A, Fletcher P. Parkinsonism hy- pyrexia syndrome in a Parkinson’s dis- RG. A neuroleptic malignant like syn- perpyrexia syndrome caused by abrupt ease patient while actively treated with drome due to levodopa therapy with- withdrawal of ropinirole. Br J Hosp Med deep brain stimulation. Mov Disord. 22, drawal. JAMA. 254, 2792-2798 (1985). (Lond). 74, 698-699 (2013). 148-149 (2007). Factor S. In: Drug induced movement Urasaki E, Fukudome T, Hirose M, Akcali A, Savas L. Malignant syn- disorders. 2nd ed. Factor S, Lang A, et al. Neuroleptic malignant syndrome drome of two Parkinson patients due Weiner W, editors. Mount Kisco, NY: (parkinsonism-hyperpyrexia syndrome) to withdrawal of drugs. Ann Acad Med Blackwell Publishing. 174-212 (2005). after deep brain stimulation of the sub- Singapore. 37, 77-78 (2008). Landriel EG, Ajler P, Ciraolo C. thalamic nucleus. J Clin Neurosci. 20, Meagher LJ, McKay D, Herkes GK, 740-741 (2013).

Clin. Pract. (2020) 17(2) 10.4172/clinical-practice.1000416 1465