Endocrine Disorders and the Neurologic Manifestations

8/17/2019 Endocrine Disorders and the Neurologic Manifestations

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Endocrinedisorders and

The neurologicmanifestations

By:

Rasyah EsiNeurologist


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Introduction

Early diagnosis and treatment of hormonalimbalance can rapidly relieve the neurologic

symptoms.

Endocrine dysfunctions various neurologicmanifestations, such as:

Headache yopathy

!cute

encephalopathyincluding coma.


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Hypothalamic"pituitarysystem

The nervous system and theendocrine system work together

to #eep the body to functionregularly.

The hypothalamic control to thesecretion of pituitary hormones Nervous system and the endocrinesystems:• maintain homeostasis• regulated secretion of hormones,


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Neurons releaseneurotransmitters

andneuromodulators at

synapses.

Neurosecretory

cells secretesubstances directly

into thebloodstream

$hormones%

neurohypophysealand

hypophysiotropiccells.



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Hypothalamus: involved inmaintaining homeostasis.

receive sensoryinputs from the

external andinternal

environment:light, nociception,

temperature,

blood pressure,blood osmolality,and blood glucose

levels


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Hypothalamus providescoordinated responsesto the pituitary glands,cerebral corte&, brain stemand spinal cord, and

sympathetic andparasympatheticpreganglionic neurons

to maintain homeostasis

through the coordinatedendocrine, behavioral,and autonomicresponses


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*. Headache

Idiopathic intracranialhypertension $pseudotumorcerebri syndrome, 'T+%:•Elevated intracranial pressure $normalbrain parenchyma and cerebrospinal -uid%•Headache, vomiting, vision changes,

abducens nerve palsy, and papilledema•nly irritability, somnolence, or apathy$young children%•/ntreated optic atrophy and vision 0ill

be lost rapidly


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'T+

It may occur associated 0ith various endocrine

disorders $adrenal insu1ciency, diabetic #etoacidosis on

Thee&act

mechanism of

'T+ isunclear


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'T+

heldon et al.

$23*4%:• pediatric 'T+ is a neuroendocrinedisorder metabolic and

hormonal derangements(hypothalamic"pituitary"adrenal a&is, renin"angiotensin"aldosterone, gro0th hormone $5H%and insulin"li#e gro0th factor"* $I56"*%,hypothalamic"pituitarythyroid a&is,

hypothalamic"pituitary"gonadal a&is, and theposterior pituitary and antidiuretic hormone%


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2. !ltered mentality

! commonpresentation in

the emergencydepartment, andcan be caused byendocrineemergencies

!ttention,a0areness, andconsciousness interactionbrainstem

reticular core,the thalamus,and thecerebral cortex

Impairedconsciousness signi(cantalteration in thewakefulnessand theawareness ofself and of the

environment


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6ind the underlying causesand to promptly stabili7e thevital signs• The causes of coma: infectious or

in-ammatory origin, structurallesions, and metabolic, to&ic ornutritional conditions

Metabolic or endocrine

encephalopathy

considered as a di)erentialdiagnosis in the patients

presenting no focalneurologic signs and no

meningeal irritation signs


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Diabetic coma

Diabetic ketoacidosis (DKA andhyperglycemic hyperosmolar syndrome

!ypoglycemia, cerebral edema follo0ing thetreatment of 89! altered mentality rangingfrom general 0ea#ness, lethargy, irritability to

coma


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!yponatremic hypovolemic adrenalcrisis in the patients 0ith underlying congenitaladrenal hyperplasia $+!H%, adrenal hypoplasiacongenita, familial glucocorticoid de(ciency, oradrenocorticotropic hormone $!+TH% de(ciency can

lead to altered mentality

!ypocalcemic tetany or sei7ure 0ith or 0ithoutunderlying hypoparathyroidism may also result inimpaired consciousness

!pathy, delirium, or psychosis can happen at serumcalcium concentrations greater than ** mgd;


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+hec#ing the

level of

blood glucose +alcium electrolytes

should beconsidered in the

(rst step of

evaluatingaltered mentality.


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"evere hyperthyroidism andhypothyroidism may be the rare cause ofaltered mentality

thyroid storm and hypothyroid coma befatal if not treated properly

Behavioral and cognitive changes,myoclonus, sei7ures, psychosis, involuntarymovements, and even coma


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!ypothyroid coma:history of thyroid

disease andprogressive lethargy0ith hypothermia,

bradycardia,constipation,

dyspnea, yello0 anddry s#in, large

tongue, and rarelysei7ure.

#hyroid storm:

fever,tachyarrhythmia,

tachypnea, dyspnea,congestive heartfailure, diarrhea,

nausea, vomiting,

and hyperhidrosis

In addition, tremors,an&iety, confusion,

delirium up tocomatose state may

develop.


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Thyroid functionshould be evaluated inthe management ofdiabetic #etoacidosis.

!ashimoto$sencephalopathy can

be developed in thepatients 0ithautoimmune thyroiddiseases and ischaracteri7ed by a

steroid-responsiveencephalopathy 0iththe presence ofantithyroid antibodies


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4. ovement disorders

ovement disorders are thedisorders causing involuntary

movements such as chorea,athetosis or tremor

any abnormal movementsare paro&ysmal or intermittent


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4. ovement disorders

#remor, an involuntary oscillating movement 0ith a (&ed fre>uency,may occur physiologically but hyperthyroidism should be considered as a

potential cause

Athetosis, a slo0 and 0rithing movement of the limbs, is oftenassociated 0ith chorea.

+horeoathetosis can occur in hyperthyroidism, !ddison disease,hypernatremia, hypocalcemia and hypoparathyrodism

*horea, a rapid repetitive movement a)ecting any part of the body, isneither rhythmic nor stereotyped and can occur in hyperthyroidism


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?. 8evelopmental delay

In 5H8 $gro0th hormonedisease%

• lo0er cognitive scales• 0hite matter abnormalities inthe corpus callosum and

corticospinal tract• reduced volumes in thalamus,hippocampus, and globus

pallidus=ebb E! et al. E)ect of gro0th hormone de(ciency on brain structure, motorfunction and cognition. Brain. 23*2@*


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Thyroid hormone regulates neuronal migration,di)erentiation, and myelination includingcerebellar development

Transgenic mouse models sho0ed: thyroid gland

dysgenesis or decreasing levels of thyroidhormones in brain tissue 0as associated 0ithcerebellar phenotypes ata&ia, impairedbalance and coordination, and reducedlocomotor activity


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+ongenital

hypothyroidismresulting from

thyroid dysgenesisor

dyshormonogenesisdelayed

development

&arly diagnosisand replacement

of thyroidhormone are

critical for afavorable outcome.


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*hildhood *ushing syndrome: cognitive impairment andbehavioral abnormality

&xcessive replacement of glucocorticoids in childrenduring the treatment of adrenal insu1ciency: delayedmyelination and brain atrophy

*ongenital adrenal hyperplasia+*A!: have 0hite matterabnormalities, temporal lobe atrophy or hippocampaldysgenesis probably produced by the disease and itstreatment. ental retardation, tremor, asymmetric tendonre-e&es, and cerebellar syndrome 0ere found in some patients

0ith +!H


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5hetti et al.

+hildren 0ith8iabetic

#etoacidosis89! history had

signi(cantly

• lo0er rates of

accuratememory andthe spatialtas#s

•

Theyconcluded:89! disruptsmemoryfunction andemphasi7edtheimportance of89!

prevention.5hetti et al $23*3%. 8iabetic #etoacidosis and memory dysfunction inchildren 0ith type * diabetes. D 'ediatr. 23*3@*?A:*3**4


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Neurologic (ndings in speci(c

endocrine disorders

*. Hypothalamic"pituitary dysfunction

Endocrine symptoms often precede neuro"ophthalmic

symptoms.

+ohort study of *CA patients 0ith hypothalamic"pituitarylesions abnormal body mass inde& or decreased gro0thvelocity occurred in twothirds of patients before the

presentation of neuro"ophthalmic symptoms

ore cautious approach to identify the etiology of endocrinesymptoms for earlier diagnosis of hypothalamicpituitary lesions

Taylor , +outo"ilva !+, !dan ;, Trivin +, ainte"Rose +, Ferah , et al. Hypothalamic"pituitary lesions in pediatricpatients: endocrine symptoms often precede neuroophthalmic presenting symptoms. D 'ediatr. 23*2@*A*:G??GA


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*. Hypothalamic"pituitary

dysfunction

!cute endocrine changes are commonly foundafter traumatic brain inury , including changes inhypothalamicpituitaryadrenal axis andantidiuretic hormone production and release

In the long term temporary and permanentalterations in pituitary function

Routine monitoring at least * year after inury forearly detection of hormonal imbalance to permitnormal gro0th and development


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2. Thyroid disorders

In congenitalhypothyroidism:

mental retardation,hypotonia,

constipation,somnolence, apnea,large fontanels, and

sensorineural hearingloss

9ocher"8ebre"emelaigne

syndrome ischaracteri7ed by

generali7ed muscularhypertrophy,

predominating in the

calf musclesassociated 0ithhypothyroidism

yopathy ofhypothyroidism in

infancy and childhoodis featured by lo0er

e&tremity orgenerali7ed muscular

hypertrophy,my&edema, and short

stature.


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!c>uiredhypothyroidism canaccompany psychosis,sei7ures, ata&ia, and

coma

Immune"mediatedmechanisms and

vasculitisaccompanying

cerebralhypoperfusion may

result in Hashimotosencephalopathy.


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5raves disease presentsneurologic features$emotional instability, an&iety,irritability, restlessness, andinattentiveness%

yopathy including muscular0ea#ness or cramp, ocularsymptoms $lid lag,impairment of convergence,or ophthalmoplegia% andmovement disorders $tremor,chorea%

Thyroto&ic periodic paralysis

or myasthenia gravis rarelyoccur in hyperthyroidism

evere thyroto&icosis may

result in a change inconsciousness


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4. !drenal disorders

'rimary adrenal insu1ciency includes

• +!H• !'• !+TH resistance• adrenoleu#odystrophy• adrenal hypoplasia congenita• adrenal hemorrhage• I!5e $intrauterine gro0th restriction, metaphyseal dysplasia,

adrenal hypoplasia congenita, and genital anomalies%syndrome

Hypotension, hyperpigmentation, hyponatremia,hyper#alemia, and hypoglycemia are presentingsigns


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4. !drenal disorders

!drenal insu1ciency presence of a

combination of chronicor subacute clinical

symptoms includingchronic fatigue,

muscle weakness,anorexia, nausea,

vomiting, diarrhea,hypotension, weightloss, headache and

skinhyperpigmentation

"erum A*#!,

aldosterone orplasma renin activitycan be included in theinitial step evaluating

suspected primaryadrenal insu1ciency.


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?. 8iabetes mellitus

• !gitation,confusion,lethargy,headache, andvomiting

8iabetic#etoacidosis andcerebral edema

• tremor,confusion,behavioralchanges,

blurred vision,sei7ure, andcoma

In hypoglycemia


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tro#e patients 0ith

hyperglycemia and 89! are prone tothrombosis

To suspect the possibility of thedevelopment of thrombosis, infection or

hemorrhage 0hen focal neurologicalde(cits are apparent or neurologicallydeteriorated in the patients 0ith 89!


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ymptomaticdiabetic neuropathy$8N'% it can be

detected throughneurophysiologicalstudies


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+onclusions

Endocrine disorders can be manifestedby various neurologic symptoms andsigns ranging from headache,

myopathy to acute encephalopathyincluding coma.

It is valuable to thin# about endocrinedisorder as the cause of theneurologic impairment.


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+onclusions

Early diagnosis and treatment of hormonalimbalance may rapidly relieve the neurologicsymptoms.

Better understanding of the interaction bet0eenthe endocrine system and the nervous system,

#no0ledge about the pathophysiology of theneurologic symptoms and signs presented in theendocrine disorders earlier diagnosis andbetter treatment of the endocrine disorders.


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Endocrine Disorders and the Neurologic Manifestations

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