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lo
euro gical Khair, et al. J Neurol Disord 2015, 3:3
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Journal
Abstract
Background: Hurst disease is the rarest yet the most fatal form of acute demyelinating encephalomyelitis.
There around 100 cases around the world, 10 of them are pediatric patients. The disease is quite severe in course
and aggressive therapy is crucial to help improving the outcome. We are reporting a rather successful story of early
recognition and treatment of young toddler with using all lines of known therapies simultaneously and aggressively.
Case report: We are reporting 2 ½ years old girl who was previously healthy. She has some viral prodromal
illness ended by severe encephalopathy. She has found to have novel H1N1 infection. Early plasma exchange and
therapeutic hypothermia have been simultaneously utilized. There was obvious significant fast improvement in clinical
status thereafter.
Discussion: Immune therapy is advised in treating Hurst disease. One trial of hypothermia has been suggested in
one case report. However, Combination of early use of plasma exchange and therapeutic hypothermia has not been
reported in literature up to our knowledge. Our patient showed impressive clinical progress following these rarely used
procedures.
Conclusion: Recognition of autoimmune encephalitis especially the most severe forms is a must. If the patient shows
enough clinical and radiological signs suggestive of Hurst disease, then aggressive immunotherapy and therapeutic
hypothermia should be considered. A more favorable outcome might be reached using these therapies simultaneously.
Keywords: Hurst disease; Acute Demyelinating Encephalomyelitis serum electrolytes, liver function tests, coagulation profiles, blood and
(ADEM); H1N1; Autoimmune encephalitis; Immunotherapy; IVIG urine cultures were all negative. Her respiratory secretions were tested
positive for H1N1 PCR using direct florescent assay (DFA), thus she
Introduction received a five days course of oral oseltamavir. Cerebro-Spinal Fluid
Hurst disease is a very rare disease affecting both adults and (CSF) analysis was done twice showing high protein but negative for
children. The disease has many parallel names used simultaneously. The bacteria and common viruses.
full name is acute hemorrhagic leuko-encephalitis (AHL, or AHLE). After three days of pediatric intensive care unit (PICU) stay, she
Occasionally it is called acute necrotizing encephalopathy (ANE), was progressive deteriorating and her respiratory support requirements
acute hemorrhagic encephalomyelitis (AHEM), or acute necrotizing were increasing. She needed inotropic support to maintain adequate
hemorrhagic leuko-encephalitis (ANHLE). The disease is often named blood pressure and perfusion. The possibility of autoimmune
“Hurst Disease” or “Weston-Hurst syndrome”, in regard to “E. Weston encephalitis was then entertained as the most likely diagnosis. Magnetic
Hurst” who has been the first author describing this disease in 1941 [1]. resonance imaging MRI of the brain was then arranged. The MRI
AHLE is considered the most severe fulminant form of acute showed very unique and characteristic features of micro-hemorrahges
disseminated encephalomyelitis (ADEM). It is thought that it in the thalamic, hippocampi, cerebellar hemispheres, pons, and cortex,
represents around 2% of cases of ADEM [2]. The disease is very rare consistent with acute hemorrhagic leukoencephalitis (AHLE/ Hurst
and tends to affects adults more than children [3]. The disease has been disease).
reported in elderly patients as well [4]. There is no significant gender On the third day of illness and the idea of autoimmune encephalitis
predominance. was the most probably diagnosis, so aggressive immunotherapy
course was started. She received five days of high dose pulse steroids
Case Report (methylprednisilone), five sessions of plasma exchange then two days
We are reporting a 2½ years old toddler girl who has been of immunoglobulins (IVIG). She also received trial of therapeutic
previously healthy with no past medical history of note. She was born
at term to non-consanguineous parents and required no prior medical
attention. She presented to the pediatric emergency center with history *Corresponding author: Dr. Abdulhafeez Mohamed Khair, Specialist & clinical
of fever and flu like symptoms for 3 days. She started to be progressively Fellow, Section of Pediatric Neurology, Hamad medical Corporation, P O Box 3050, Doha,
Qatar, Tel: 0097433165827; E-mail: drabboody@hotmail.com, akhair1@hmc.org.qa
sleepy then she developed a sudden generalized tonic clonic seizure for
five minute. Her initial assessment showed Glasgow coma scale (GCS) Received May 09, 2015; Accepted May 23, 2015; Published May 26, 2015
of 8/15 with otherwise reactive pupils and no focalizing neurological Citation: Khair AM, Elsotouhy A, Batool M, Elsaid M (2015) Acute Hemorrhagic
signs. She was subsequently intubated and mechanically ventilated. The Leukoencephalitis (Hurst Disease) Secondary to H1N1 in a Child - A Story of Full
Recovery from Qatar, A Case Report. J Neurol Disord 3: 231. doi: 10.4172/2329-
initial impression was acute meningio-encephalitis, so broad spectrum 6895.1000231
antibiotics and antiviral drugs were commenced and lumber puncture
Copyright: © 2015 Khair AM, et al. This is an open-access article distributed under
was performed after some period of stabilization. the terms of the Creative Commons Attribution License, which permits unrestricted
use, distribution, and reproduction in any medium, provided the original author and
Her initial complete hemorgam counts, inflammatory markers, source are credited.
J Neurol Disord
ISSN: 2329-6895 JND, an open access journal Volume 3 • Issue 3 • 1000231
Citation: Khair AM, Elsotouhy A, Batool M, Elsaid M (2015) Acute Hemorrhagic Leukoencephalitis (Hurst Disease) Secondary to H1N1 in a Child - A
Story of Full Recovery from Qatar, A Case Report. J Neurol Disord 3: 231. doi: 10.4172/2329-6895.1000231
Page 2 of 4
Figure 1: 1) Axial Non-Contrast CT examination of the brain at the level of the Figure 4: Axial Susceptibility weighted images (SWI) MRI of the brain at the
pons (A), Basal Ganglia and Thalami (B) showing subtle hypodensity along level of the pons (A), Basal Ganglia and Thalami (B) showing patchy dark
the thalami with no gross basal ganglia or posterior fossa changes. blooming low signal intensity denoting parenchymal hemorrhages as well.
Figure 5: Axial non-contrast T1W1 MRI of the brain at the level of the pons
(A), Basal Ganglia and Thalami (B) showing subtle hypointensity along the
thalami with no gross basal ganglia or posterior fossa changes.
J Neurol Disord
ISSN: 2329-6895 JND, an open access journal Volume 3 • Issue 3 • 1000231
Citation: Khair AM, Elsotouhy A, Batool M, Elsaid M (2015) Acute Hemorrhagic Leukoencephalitis (Hurst Disease) Secondary to H1N1 in a Child - A
Story of Full Recovery from Qatar, A Case Report. J Neurol Disord 3: 231. doi: 10.4172/2329-6895.1000231
Page 3 of 4
J Neurol Disord
ISSN: 2329-6895 JND, an open access journal Volume 3 • Issue 3 • 1000231
Citation: Khair AM, Elsotouhy A, Batool M, Elsaid M (2015) Acute Hemorrhagic Leukoencephalitis (Hurst Disease) Secondary to H1N1 in a Child - A
Story of Full Recovery from Qatar, A Case Report. J Neurol Disord 3: 231. doi: 10.4172/2329-6895.1000231
Page 4 of 4
therapeutic plasma exchange in the management of acute hemorrhagic to dramatic recovery in acute hemorrhagic leukoencephalitis. J Child Neurol
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15. Ichikawa K, Motoi H, Oyama Y, Watanabe Y, Takeshita S (2013) Fulminant
13. Meilof JF, Hijdra A, Vermeulen M (2001) Successful recovery after high-dose form of acute disseminated encephalomyelitis in a child treated with mild
intravenous methylprednisolone in acute hemorrhagic leukoencephalitis. J hypothermia. Pediatr Int 55: e149-151.
Neurol 248: 898-899.
16. Vargas WS, Merchant S, Solomon G (2012) Favorable outcomes in acute
14. Payne ET, Rutka JT, Ho TK, Halliday WC, Banwell BL (2007) Treatment leading necrotizing encephalopathy in a child treated with hypothermia. Pediatr Neurol
46: 387-389.
J Neurol Disord
ISSN: 2329-6895 JND, an open access journal Volume 3 • Issue 3 • 1000231