Endocrine Disorders and Its Neurologic Manifestations

ENDOCRINE DISORDERS AND ITS
NEUROLOGIC MANIFESTATIONS
Dr Ratnesh Singh Kanwar

Sc “F” & Joint Director
Div of Health Endocrinology & Thyroid Research
INMAS, DRDO, Delhi 110054
INTRODUCTION
• Both the nervous system and the endocrine system
are involved intimately in maintaining homeostasis.
• Therefore, endocrine dysfunctions may lead to

various neurologic manifestations, which can occur
in any endocrine disorders including disorders of
pituitary gland, thyroid, parathyroids, pancreas,
adrenal glands, and gonads.
• It is beneficial to know the neurologic signs and

symptoms caused by the endocrine disorders in
diagnosing as well as managing endocrine disorders.
HYPOTHALAMIC-PITUITARY SYSTEM
• It is important to understand the hypothalamic-pituitary
system and the intimate relation between nervous system and
endocrine system.
• The neuroendocrine system which is made up of the nervous

system and the endocrine system work together to keep the
body to function regularly.
• It focuses on the hypothalamic control to the secretion of
pituitary hormones, but the broad concept includes multiple
reciprocal interaction between the nervous system and the
endocrine systems to maintain homeostasis and to respond
properly to environmental stimuli through the regulated
secretion of hormones, neurotransmitters, or
neuromodulators.
• Neurons release their neurotransmitters and neuromodulators
at synapses.
HYPOTHALAMIC-PITUITARY SYSTEM
• Neurosecretory cells secrete substances directly into the
bloodstream to act as hormones. They include
neurohypophyseal and hypophysiotropic cells.
• Hypothalamus is the ultimate brain structure involved in
maintaining homeostasis.
• It has many specified nuclei which receive sensory inputs from
the external and internal environment such as light,
nociception, temperature, blood pressure, blood osmolality,
and blood glucose levels.
• Many hormones also exert both negative and positive
feedback directly on the hypothalamus.
• Hypothalamus provides coordinated responses to the pituitary
glands, cerebral cortex, brain stem and spinal cord, and
sympathetic and parasympathetic pre-ganglionic neurons to
maintain homeostasis through the coordinated endocrine,
behavioral,and autonomic responses.
SPECIFIC NEUROLOGIC MANIFESTATIONS:
POTENTIAL ENDOCRINE DISORDERS
• Headache
• Altered mentality
• Abnormal muscle strength, muscle tone and gait
• Movement disorders
• Developmental delay
HEADACHE
• Headache may be a nonspecific sign, but it can be caused by pathologic conditions
including idiopathic intracranial hypertension.
• Idiopathic intracranial hypertension (pseudotumor cerebri syndrome, PTCS) is the

presence of elevated intracranial pressure in the setting of normal brain parenchyma
and cerebrospinal fluid.
• Headache, vomiting, vision changes, abducens nerve palsy, and papilledema are
commonly presented.
• If it is untreated, it may progress to optic atrophy and vision will be lost

rapidly.Therefore, early diagnosis and treatment are crucial.
• The exact mechanism of PTCS is unclear, but it may occur associated with a variety of
conditions, including various endocrine disorders such as adrenal insufficiency, diabetic
ketoacidosis on treatment, hyperadrenalism, hyperthyroidism, and
hypoparathyroidism.
• PTCS is a neuroendocrine disorder, illustrating many metabolic and hormonal

derangements within the hypothalamic-pituitary-adrenal axis, renin-angiotensin-
aldosterone, growth hormone (GH) and insulin-like growth factor-1 (IGF-1),
hypothalamic-pituitarythyroid axis, hypothalamic-pituitary-gonadal axis, and the
posterior pituitary and antidiuretic hormone.
ALTERED MENTALITY
• Altered mental status is a common presentation and can be caused by
endocrine emergencies.
• Attention, awareness, and consciousness can be maintained by the interaction among the brainstem
reticular core, the thalamus, and the cerebral cortex.
• Impaired consciousness means a significant alteration in the wakefulness and the awareness of self and
of the environment.
• It is important to find the underlying causes and to promptly stabilize the vital signs. The causes of coma
can be classified with infectious or inflammatory origin, structural lesions, and metabolic, toxic or
nutritional conditions.
• Metabolic or endocrine encephalopathy should be considered as a differential diagnosis in the patients

presenting no focal neurologic signs and no meningeal irritation signs.
• Diabetic coma is one of differential diagnosis in the emergency room in managing patients with altered
mentality.
• Diabetic ketoacidosis (DKA) and hyperglycemic hyperosmolar syndrome are the most serious acute
hyperglycemic emergency). Hypoglycemia, cerebral edema following the treatment of DKA may also be
presented with altered mentality ranging from general weakness, lethargy, irritability to coma.
• Hyponatremic hypovolemic adrenal crisis in the patients with underlying congenital adrenal hyperplasia
(CAH), adrenal hypoplasia congenita, familial glucocorticoid deficiency, oradrenocorticotropic hormone
(ACTH) deficiency can leadto altered mentality. Hypocalcemic tetany or seizure with or without
underlying hypoparathyroidism may also result in impaired consciousness
ALTERED MENTALITY
• Checking the level of blood glucose, calcium and electrolytes should be
considered in the first step of evaluating altered mentality.
• Severe hyperthyroidism and hypothyroidism may be the rare cause of altered

mentality, thyroid storm and hypothyroid coma, respectively. They could be
fatal if not treated properly.
• Behavioral and cognitive changes, myoclonus, seizures, psychosis, involuntary

movements, and even coma may occur.
• In hypothyroid coma, there may be a history of previous thyroid disease and

progressive lethargy with hypothermia, bradycardia, constipation, dyspnea,
yellow and dry skin, large tongue, and rarely seizure.
• In thyroid storm, fever, tachyarrhythmia, tachypnea, dyspnea, congestive

heart failure, diarrhea, nausea, vomiting, and hyperhidrosis can be
manifested.
• Hashimoto’s encephalopathy can be developed in the patients with

autoimmune thyroid diseases and is characterized by a steroid-responsive
encephalopathy with the presence of antithyroid antibodies.
ABNORMAL MUSCLE STRENGTH
• Muscle weakness, pain, and stiffness are common symptoms of endocrine disorders. Systemic
characteristic symptoms of specific endocrine disorders usually precede the onset of weakness, but
muscle weakness may be the initial symptom.
• Endocrine myopathy should be considered as one of the etiology of muscle weakness, because
specific treatment is available in endocrine myopathy. Thyroid dysfunction (hyper- or
hypothyroidism), parathyroid disorders (hyper- or hypoparathyroidism), and adrenal diseases
(Cushing disease, Addison disease, or hyperaldosteronism) may cause endocrine myopathies.
• Pointed that clinical features of most of endocrine myopathies in childhood are usually
characterized by the presence of proximal muscle involvement such as pelvic or shoulder girdle
muscles, relatively mild morphological muscular abnormalities even in the presence of severe
clinical symptoms, and favorable outcome to treatment.
• Weakness is usually much more prominent in the legs than in the arms, and abnormal gait can be
the initial symptom of either proximal or distal leg weakness.
• Muscle stiffness and spasms occur in myotonia, dystonia, and other movement disorders, but can
be present in hypothyroidism or thyrotoxicosis when motor unit activity is continuous.
• In hypothyroidism, the stiffness gets worsen by activity and may be painful with the slowing of
muscular contraction and relaxation in performing tendon reflexes. Tone is functionally defined as
resistance to passive movement.
MOVEMENT DISODERS
•Movement disorders are the disorders causing involuntary movements
such as chorea, athetosis or tremor.
•Many abnormal movements are paroxysmal or intermittent.
•Chorea, a rapid repetitive movement affecting any part of the body, is

neither rhythmic nor stereotyped and can occur in hyperthyroidism.
•Athetosis, a slow and writhing movement of the limbs, is often associated

with chorea.
•Choreoathetosis can occur in hyperthyroidism, Addison disease,
hypernatremia, hypocalcemia and hypoparathyrodism.
•Tremor, an involuntary oscillating movement with a fixed frequency, may

occur physiologically but hyperthyroidism should be considered as a
potential cause
DEVELOPMENTAL DELAY
• The effect of GHD on brain structure, motor function, and cognition were
studied by cognitive assessment and diffusion tensor, and volumetric
magnetic resonance imaging (MRI) in children with GHD.
• In GHD, lower cognitive scales, white matter abnormalities in the corpus

callosum and corticospinal tract, and reduced volumes in thalamus,
hippocampus, and globus pallidus were found compared with controls
with idiopathic short stature.
• Thyroid hormone regulates neuronal migration, differentiation, and

myelination including cerebellar development.
• Congenital hypothyroidism resulting from thyroid dysgenesis or

dyshormonogenesis, occurring in 1 per 3,000-4,000 live births, may
present delayed development, if not treated promptly.
• Early diagnosis and replacement of thyroid hormone are critical for a

favorable outcome.
DEVELOPMENTAL DELAY
• Recently several reports describing the association between various
adrenal disorders and neurologic manifestations were published.
• Childhood Cushing syndrome can be associated with cognitive

impairment and behavioral abnormality, even after recovery from the
hypercortisolism.
• Exposure to excessive replacement of glucocorticoids in children during

the treatment of adrenal insufficiency has been associated with
neurological and MRI abnormalities including delayed myelination and
brain atrophy.
• Even though the clinical implication is unclear, several reports suggest

that patients with CAH are more likely to have white matter
abnormalities, temporal lobe atrophy or hippocampal dysgenesis
probably produced by the disease and its treatment.
• Mental retardation, tremor, asymmetric tendon reflexes, and cerebellar

syndrome were found in some patients with CAH.
NEUROLOGIC FINDINGS IN SPECIFIC
ENDOCRINE DISORDERS
• THYROID DISORDERS
• PARATHYROID DISORDERS
• ADRENAL DISORDERS
• DIABETES MELLITUS
THYROID DISORDERS
• In congenital hypothyroidism, mental retardation, hypotonia,
constipation, somnolence, apnea, large fontanels, and
sensorineural hearing loss may be presented.
• It may accompany Kocher-Debre-Semelaigne syndrome which is

characterized by generalized muscular hypertrophy,
predominating in the calf muscles associated with
hypothyroidism.
• Myopathy of hypothyroidism in infancy and childhood is

featured by lower extremity or generalized muscular
hypertrophy, myxedema, and short stature.
• Although it is possible to detect congenital hypothyroidism early

in neonate through neonatal screening test, it can rarely be
missed, requiring repeated thyroid function test at any time if
there are suspicious clinical symptoms suggesting
hypothyroidism.
THYROID DISORDERS
• In neonatal hyperthyroidism, craniosynostosis and developmental delay can
occur.
• Acquired hypothyroidism can accompany psychosis, seizures, ataxia, and coma.
• Immune-mediated mechanisms and vasculitis accompanying cerebral

hypoperfusion may result in Hashimoto's encephalopathy.
• Graves’ disease presents neurologic features such as emotional instability,

anxiety, irritability, restlessness, and inattentiveness.
• Myopathy including muscular weakness or cramp, ocular symptoms such as lid

lag, impairment of convergence, or ophthalmoplegia, and movement disorders
such as tremor or chorea can be manifested.
• Thyrotoxic periodic paralysis or myasthenia gravis rarely occur in

hyperthyroidism.
• Severe thyrotoxicosis may result in a change in consciousness, as seen in thyroid

storm.

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ENDOCRINE DISORDERS AND ITS

Dr Ratnesh Singh Kanwar

• Therefore, endocrine dysfunctions may lead to

• It is beneficial to know the neurologic signs and

• The neuroendocrine system which is made up of the nervous

• Abnormal muscle strength, muscle tone and gait

• Idiopathic intracranial hypertension (pseudotumor cerebri syndrome, PTCS) is the

• If it is untreated, it may progress to optic atrophy and vision will be lost

• PTCS is a neuroendocrine disorder, illustrating many metabolic and hormonal

• Metabolic or endocrine encephalopathy should be considered as a differential diagnosis in the patients

• Severe hyperthyroidism and hypothyroidism may be the rare cause of altered

• Behavioral and cognitive changes, myoclonus, seizures, psychosis, involuntary

• In hypothyroid coma, there may be a history of previous thyroid disease and

• In thyroid storm, fever, tachyarrhythmia, tachypnea, dyspnea, congestive

• Hashimoto’s encephalopathy can be developed in the patients with

•Many abnormal movements are paroxysmal or intermittent.

•Chorea, a rapid repetitive movement affecting any part of the body, is

•Athetosis, a slow and writhing movement of the limbs, is often associated

•Tremor, an involuntary oscillating movement with a fixed frequency, may

• In GHD, lower cognitive scales, white matter abnormalities in the corpus

• Thyroid hormone regulates neuronal migration, differentiation, and

• Congenital hypothyroidism resulting from thyroid dysgenesis or

• Early diagnosis and replacement of thyroid hormone are critical for a

• Childhood Cushing syndrome can be associated with cognitive

• Exposure to excessive replacement of glucocorticoids in children during

• Even though the clinical implication is unclear, several reports suggest

• Mental retardation, tremor, asymmetric tendon reflexes, and cerebellar

• It may accompany Kocher-Debre-Semelaigne syndrome which is

• Myopathy of hypothyroidism in infancy and childhood is

• Although it is possible to detect congenital hypothyroidism early

• Acquired hypothyroidism can accompany psychosis, seizures, ataxia, and coma.

• Immune-mediated mechanisms and vasculitis accompanying cerebral

• Graves’ disease presents neurologic features such as emotional instability,

• Myopathy including muscular weakness or cramp, ocular symptoms such as lid

• Thyrotoxic periodic paralysis or myasthenia gravis rarely occur in

• Severe thyrotoxicosis may result in a change in consciousness, as seen in thyroid

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