Case Reports in Neurological Medicine / 2018 / Article / Tab 2

Case Report

Hypothermia in Multiple Sclerosis: Beyond the Hypothalamus? A Case Report and Review of the Literature

Table 2

Review of the literature: summary of the 1st presentation of patients with hypothermia in Multiple Sclerosis (MS) and the clinical course of the disease.

ReferencesPatient detailsDisease duration;
MS-type;
EDSS
Main complaint(s) before admissionTemperature
at admission (°C)
Cognitive symptoms at admissionNew neurological signs and symptomsDysarthria and/or
dysphagia
Haematological abnormalities and onsetHyponatremia and plasma/urinary osmolalitiesNeuroimaging and/or autopsy studiesSuspected diagnosis and disease courseType of hypothermia and
(number of hypothermic episodes)

[7]61 FNK;
NK;
NK
Lethargy,
anorexia,
poor fluid intake
29.4NKNKNKHb 12.9 g/dl;
MCV 84 fl
Platelets 19 × 109/l
Bone marrow aspirate: erythroid hypoplasia
NKNoDeathAcute
(1)

[8]41 F7 years;
NK;
EDSS: 7.0
3 weeks
confusion, apathy
32.6Confusion,
Stupor
Marked rigidity in all limbsNoAfter 1/52:
Anaemia (Hb 7.9 g/dl)
Thrombocytopenia (61 × 109/l)
NoHead CT: no abnormality detectedTreated with passive rewarming. Full clinical recovery in 6 daysChronic:
(1)

[8]52 F24 years;
NK;
EDSS: 8.0
3 weeks:
confusion, lethargy
31.0ComaNoNoThrombocytopenia (50 × 109/l) at admission, peaking after 5 days
(28 × 109/l) and anaemia (Hb 7.4 g/dl)
Yes: (Na+ 107 mmol/l)
(?SIADH)
NoTreated with steroids, passive rewarming, hypertonic saline, and furosemide. Full clinical recovery in 5 daysChronic:
(1)

[9]55 F24 years;
NK;
EDSS: 6.0
1 week:
confusion, lethargy,
visual hallucinations
33.0ConfusionGeneralised myoclonus
neck stiffness
No8 days after: pancytopenia
Hb 9.2 g/dl,
Platelet 80 × 109/l,
Leukocytes 2.9 × 109/l
NoHead CT: no abnormality detected
Brain autopsy:
multiple old plaques at various locations (incl. basal ganglia, corpus callosum,
occipital white matter, and right upper cerebellar peduncle).
No hypothalamic lesions except some recent axon swellings and cell loss
Developed bronchopneumonia. Treated with antibiotics. Full clinical recoveryAcute:
(4)

[9]55 F22 years;
NK;
NK
4 weeks: confusion,
bradyphrenia, incontinence
NKNKAugmented paraparesisNoNoNoBrain MRI and CT performed after the 2nd admission with hypothermia. Brain MRI and head CT: Important lesions in periventricular and posterior part of corpus callosum
No hypothalamic lesions
Developed bronchopneumonia. Treated with antibiotic and passive rewarming. Full clinical recovery.Acute:
(2)

[9]58 M16 years;
NK;
EDSS: 7.0
2 weeks:
lethargy, dysarthria, dysphagia
<35Memory deficitTetraparesis, bilateral central nystagmusDysarthria, dysphagiaThrombocytopenia: 100 × 109/lNoBrain MRI performed after the 4th admission with hypothermia.
Brain MRI: several periventricular plaques.
No hypothalamic lesions
Developed bronchopneumonia. Treated with antibiotics and passive rewarming recovered in days. Some motor deterioration remainedAcute:
(4)

[10]52 M14 years;
NK;
NK
Augmented motor deficits32.8ConfusionAugmented motor paresisNoNoYes: (Na+ 114 mEq/l), plasma hyposmolarity
(? SIADH)
Brain MRI:
No hypothalamic lesions
Treatment with NaCl infusion and fluid restrictions. Hypothermia self-resolved. Clinical full recoveryAcute:
(3)

[11]63 F25 years;
NK;
NK
Visual disturbances, depression paranoid, unable to stand up without help32.4ConfusionWorsening neurological signs: bilateral Babinski sign, paraparesis,
paresthesia, and ataxia in the right arm and mild postural tremor
DysarthriaDeranged LFTs (ALT and AST mildly raised with hypoalbuminaemia, 31.7 g/l)NoBrain MRI: diffuse white matter lesions No hypothalamic lesionsTreated with passive rewarming and parenteral thiamine (? Wernicke Encephalopathy) Normothermia in 3 weeksAcute
(1)

[11]68 F32 years;
NK;
NK
3 weeks:
gait abnormalities,
dysarthria
31.6Confusion, drowsinessSevere paraparesis,
bilateral Babinski sign, asterixis, partial right lateral rectus palsy, cerebellar signs
DysarthriaSevere hypoalbuminaemia (18.9 g/l), decreased folic acidNoBrain MRI: multiple periventricular lesions.
No hypothalamic lesions
Treated with parenteral thiamine (? Wernicke Encephalopathy). Full recovery within 1 monthAcute
(1)

[12]53 FNK;
NK;
NK
5 days: lethargy, dysphagia, dysarthria29.0ConfusionSpastic tetraparesis with bilateral extensor plantar but depressed reflexesDysarthriaThrombocytopenia: platelets 79 × 109/l
Increased APTT
?DIC
Raised amylase (321 IU/l)
No.Brain and spine autopsy: multiple plaques in the brain and spinal cord. A large hypothalamic plaque was found with evidence of current activity and demyelinationPassive rewarming, antibiotics, and atropine. Developed bronchopneumonia, pancreatitis, and diedAcute
(1)

[13]44 F10 years;
PR-MS;
NK
Few days: confusion,
disorientation, hallucinations
33.3ConfusionFlaccid paraplegia and cerebellar syndrome (not augmented)NoNoNoBrain MRI: T2W hyperintensities in the periventricular white matterPassive rewarming and full clinical recovery within 10 daysNK

[14]48 M5 years;
NK;
EDSS: 6.0
3 weeks: confusion, disorientation, dysarthria deteriorating mobility, drowsiness,
cold lower extremities
Initially 36.0 then 31.0StuporInitially flaccid paraparesis and increased tone in the upper limbs. Deterioration over 48 h. He developed repetitive facial twitching, neck stiffness, left lower motor facial weakness, and decerebrate posturingDysarthriaThrombocytopenia: 27 × 109/l
Anaemia: Hb 12.7 g/dl.
Increased PT and APTT and low folate
NoCT head: moderate brain atrophy.
Previous MRIs had been normal. Brain MRI: after 1st and 2nd admissions with hypothermia:
multiple high signals in periventricular white matter. No hypothalamic lesions.
Brain autopsy: plaques in periventricular, midbrain, pons, medulla and hypothalamus (incl. posterior hypothalamic nucleus)
Initially treated with IV methylprednisolone for MS relapse, then with antibiotics for ?UTI. Then, passive rewarming. Normothermia after further 48 h. Packed cells, platelets, and plasma proteins transfusion for bleeding. Discharged in 30 days. Residual spastic paraparesis, incoordination, mild upper limb weakness, and sensory deficit after T12Acute then chronic:
(2)

[14]59 M30 years;
NK;
EDSS: 7.0
4 weeks: increasing fatigue, lethargy,
confusion, then drowsiness, dysphagia, and dysarthria
33.0StuporNKDysphagia, dysarthriaThrombocytopenia 95 × 109/lNoNKPassive rewarming and IV fluids. Normothermia in 36 hours. Paranoid psychosis and confusion, MI and severe LVF. Residual cognitive impairmentAcute
(2)

[14]57 F20 years;
NK;
EDSS: 7.0
Decreased mobility, lethargy, dysphagia,35.0Oriented (initially)Bilateral optic atrophy and absent oculocephalic response, neck stiffness, rigidity, spastic tetraparesisDysarthria, dysphagiaThrombocytopenia. when normothermic (141) then 99 × 109/l.
Raised APTT time.
Raised platelets antibodies
Yes: (Na+ 130 mmol/l)Head CT: bilateral periventricular low density lesionsRewarming, IV fluids and IV methylprednisolone and antibiotics for ?UTI and respiratory infections were given. Normothermia within 24 hours. Full clinical recovery in 4 weeksAcute:
(2)

[14]64 F30 years;
NK;
EDSS: 9.0
Deterioration of motor function, speech disturbance, peripheral oedema, fluctuating consciousness34.7ConfusionPeriorbital oedema, augmented tetraparesis, impaired palatal movementsDysarthriaNoYes: (Na+ 130 mmol/l) corrected with fluid restriction. Normal plasma and urinary osmolalitiesNKPassive rewarming. Normothermia in 24 hours. Full clinical recovery in 7 daysAcute
(1)

[14]47 FNo previous MS (diagnosed in retrospective);
EDSS: 3.0
Withdrawal and lethargy29.0ComaNeck stiffness, generalised hypertonia.
After 3 days: bilateral extensor plantar responses, mild paraparesis, optic disc pallor
NKThrombocytopenia: 33 × 109/l
Anemia: Hb 10.2
NoBrain MRI: diffuse cortical atrophy, T2W hyperintense periventricular lesions. No hypothalamic lesionsRewarming. Normothermia in 3 days.
Residual physical and cognitive deficits
Acute:
(2)

[15]NK FNK;
NK;
NK
Motor and cognitive declineNKDrowsinessAugmented flaccid paresisDysarthriaThrombocytopeniaNKHead CT and brain MRI: No hypothalamic lesionsNKChronic with acute episode:
(2)

[15]NK FNK;
NK;
NK
Motor and cognitive declineNKDrowsinessAugmented flaccid paresisDysarthriaThrombocytopeniaNKHead CT and brain MRI: No hypothalamic lesions.NKChronic with acute episodes (NK)

[16]45 F28 years;
SP- MS;
EDSS: 8.0
4 weeks: hypothermia (32-33°C), stupor, hypotension, hyponatraemia, and hypoglycaemia33.4StuporNoDysarthriaChronic normocytic anaemia. Elevated APTT (61 s). Raised CRP with negative blood cultures. HypoglycaemiaYes: (124 mmol/l).
?CSW syndrome
Head CT and brain MRI: known right parietal defect (previous brain abscess), generalised atrophy, periventricular white matter lesions. particularly in the callosum and a hyperintense lesion in the septal region of right thalamus. No hypothalamic lesionsAntibiotics, IV fluids. Initially recovered then further deterioration within a week (33.1°C) stupor and severe hypotension. Within 2 weeks a 3rd episode of hypothermia (31.2°C), bradycardia, and hypotension. She was treated with droxidopa and then discharged once normothermic and stableAcute:
(6)

[4]61 F30 years;
SP-MS;
NK
Confusion, agitation33.9Confusion, agitationNoNoNoNoBrain MRI performed after 3rd hypothermic episode. Periventricular and brain stem plaques were seen with small vessel ischaemia in the ganglionic regions.
No hypothalamic involvement
Spontaneous improvement and discharge with a T of 35.2°CChronic with acute episodes
(6)

[17]41 M7 years;
NK;
NK
3 weeks: slurred speech, hypothermia, dysarthria, paranoid delusions, auditory, visual and tactile hallucinations30.0Confusion then comaBilateral facial droop, miosis, paraplegia (also present before), and bilateral upper extremities weaknessDysarthriaPlatelets: 113000/mm3No
Brain MRI: increased overall lesions and new T2W hypothalamic hyperintensityPassive rewarming. Then antibiotics and respiratory assistance for ?SUO. Full clinical recovery in 6 weeks. Five monthly IV methylprednisolone infusions (1 g/month)Acute
(6)

[18]39 M24 years;
SP-MS;
EDSS: 6.5
Few weeks: augmented spasticity, cognitive decline,
confusion
31.0StuporSpastic tetraparesisDysarthria,
dysphagia
Thrombocytopenia (75 × 109/l)
Leukopenia (0.7 × 109/l)
Elevated APTT (37 s)
Raised ALT and AST
NoBrain MRI: T2W multiple white matter lesions and atrophy of corpus callosum.
Hypothalamic involvement with bilateral nonenhancing preoptic lesions.
After 1 year MRI showed no longer signs
Full clinical recoveryNK

[18]49 M32 years;
PP-MS;
EDSS: 7.5
Confusion32.4Psychomotor slowingAugmented tetraparesis, bilateral pyramidal syndrome, right cerebellar syndromeDysarthria, dysphagia
Thrombocytopenia (79 × 109/l)
Raised AST and ALT
NoBrain MRI: T2W white matter lesions. No hypothalamic involvementAntibiotics for sepsis
and 3 steroid injections. Full clinical recovery within days
Acute
(1)

Expanded Disability Status Scale (EDSS); Primary Progressive MS (PP-MS); Secondary Progressive MS (SP-MS); not known (NK); aspartate transaminase (AST); alanine transaminase (ALT); disseminated intravascular coagulation (DIC); activated partial thromboplastin time (APTT); Syndrome of Inappropriate Anti-Diuretic Hormone (SIADH); mean corpuscular volume (MCV); T-2 weighted (T2W).