HYPOTHALAMUS

AND ITS CONNECTIONS

(APPLIED)

RAMA BISWAS
MD,Final
Part Student
Department of Neurology
Dhaka Medical College Hospital
 Contents
• Diseases of Hypothalamus
• Effects of Hypothalamic disease.
-Dysfunction
.Hypofunction
.Hyperfunction
-Mechanical effect
• Brief description of some relevant diseases
 Diseases of hypothalamus
Classification
 Developmental disorders
 Midline cleft defects
 Kallman’s syndrome
 Septo-optic dysplasia
 Infiltrative and inflammatory diseases
 Sarcoidosis
 Tuberculosis
 Langerhans cell histiocytosis
 Diseases of hypothalamus
Classification (cont.)
 Space occupying lesions
 Craniopharyngioma
 Glioma
 Meningioma
 Hemangioblastoma
 Hamartoma
 Cysts : Epidermoid, dermoid, colloid
 Metastasis
 Lymphoma and leukaemia
 Diseases of hypothalamus
Classification (cont.)
 Vascular
 Stroke
 Arterio-venous malformation
 Aneurysm
 Functional abnormalities
 Isolated hormone or receptor defect
 Systemic illness and psychosocial deprivation
 Diseases of hypothalamus
Classification (cont.)
 Iatrogenic
 Parasellar surgery
 Cranial irradiation

 Miscellaneous
 Hydrocephalus
 Pituitary adenoma
 Wernicke’s encephalopathy
 Effects of hypothalamic diseases
Dysfunction
 Endocrine
 Autonomic regulation
 Temperature regulation
 Eating behavior
Mechanical effects
 Optic chiasma compression
 Hydrocephalus
 Endocrine dysfunctions
Hypofunction
Growth hormone deficiency
Hypothalamic hypogonadism
Secondary hypothyroidism
Central diabetes insipidus
Pituitary insufficiency
 Endocrine dysfunctions (cont.)
Hyperfunction

 Hypothalamic precocious puberty

 Hypothalamic hyperprolactinaemia
 Syndrome of inappropriate ADH secretion
 Endocrine dysfunctions (cont.)
Abnormalities of Growth:
 Deficiency or Excess of GHRH/GH
 Dwarfism/acromegaly and gigantism
 Endocrine dysfunctions (cont.)
Hypothalamic hypogonadism

Causes
 Isolated GnRH deficiency
 Structural lesion of hypothalamus
 Hyperprolactinaemia
 Endocrine dysfunctions (cont.)
Hypothalamic hypergonadism
 Structural lesion
(Hamartoma most common)
 Idiopathic
 Precocious Puberty

• Abnormally early onset of androgen secretion in

boys and estrogen secretion in girls
• Causes-
 Teratoma of pineal gland or mediastinum,
androgenic tumour of testes and adrenal- male
 Hypothalamic disease and estrogen secreting
ovarian tumours-female
 Hamartoma in both sexes ,in a number of cases
gelastic seizure have been conjoined
A 45 yrs old female with joint pain , polyuria, polydypsia
 Endocrine dysfunctions (cont.)

Cranial diabetes insipidus

Pathogenesis
• Lack of ADH
• Impaired renal water reabsorption
• Diuresis
• Raised plasma osmolarity
• Increased thirst
 Endocrine dysfunctions (cont.)
Cranial diabetes insipidus
Cause
 Parasellar surgery
 Head injury
 ICSOL
 Infiltrative granulomatous disease eg.
Neurosarcoidosis

 Drugs eg.CBZ, lithium

 Idiopathic
 Endocrine dysfunctions (cont.)
Cranial diabetes insipidus
Investigation
 Water deprivation test :
 Plasma osmolality > 300 mOsm/L
 Urine osmolality < 600 mOsm/L
> 50% improvement after administration
of DDAVP
A 63 year old man with poor appetite and Na 114 mmol/L
 Endocrine dysfunctions (cont.)

Syndrome of inappropriate ADH secretion

(SIADH)
Pathogenesis :
• Excessive ADH
• Excessive water reabsorption in collecting
tubule
• Dilutional hyponatraemia
 Endocrine dysfunctions
SIADH (cont.)

Causes-

CNS disorders :Stroke, infection, injury,tumour

Pulmonary disorders : Pneumonia, TB , CA
Drugs : Carbamazepine, TCA, Antipsychotic
Ectopic production of ADH: Carcinoma,
thymoma,mesothelioma, lymphoma
Idiopathic
 Endocrine dysfunctions (cont.)
SIADH (cont.)
Diagnosis
 Low plasma sodium (<130 mmol/L)
 Low plasma osmolality (<270 mosmol/L)
 Urine osmolality not minimally low (> 150
mosmol/L)
 Urine Na not minimally low (>30 mmol/L)
 Exclusion of other causes
 Appropriate clinical setting
A 50 yr old male with headache, confusion and serum Na 110mmol/L
 Cerebral Salt Wasting Syndrome
• Also known as Neurogenic SWS or NS
• Caused by acute intracranial diseases,
tumours and after neurosurgical procedure
• BNP and ANF are responsible
• Opposes the action of ADH in RT and also
inhibit ADH release from hypothalamus
• Sodium loss rather than water retention
• Fluid restriction may be dangerous
Distinguishing features between CSWS and SIADH
 Pituitary insufficiency
• Loss of function of the anterior pituitary gland
• Result from disease of the pituitary itself or from
hypothalamic disease
• Causes-
• Inherited
• Tumor eg. Craniopharyngioma
• Pituitary surgery
• Pituitary apoplexy
• Sheehan Syndrome
• Cranial irradiation for cerebral tumors
Pituitary apoplexy (pituitary macroadenoma with hemorrhage): Axial T1W (a), T2W (b) and GRE (c) images
reveal pituitary mass with intratumoral hemorrhage (arrow)
 Disturbance of temperature regulation

Hyperthermia and malignant Hyperthermia:

Anterior hypothalamic lesion
 Disturbance of temperature regulation

Hypothermia : Posterior hypothalamic lesion

Persistent temparature of ≤ 35°C (95°F)

Poikilothermia: Large lesion in the posterior

hypothalamus
 Disturbance of temperature regulation

Causes:
 Structural – stroke, head injury, surgery,
demyelination
 Functional – Spontaneous recurrent
hypothermia

o Shapiro’s syndrome (cholesteatoma of 3 rd V.)

o Reverse Shapiro’s syndrome
Disturbance of Autonomic regulation
• Acute autonomic crises
• Also known as Sympathetic Storm
Causes-
• Toxic and pharmacologic agents-
Cocaine
Phenylpropanolamine
TCA overdose
OP insectisides
• Severe head injury
• Hypertensive ICH
Disturbance of Autonomic regulation
• Acute autonomic crises (cont..)
Presentatoin-
i. Abrupt overactivity of sympathetic and
parasympathetic nervous system
ii. Occcasional sign of CNS excitation eg.seizure
iii.Cushing response/reflex (HTN,↓HR,Irreg.RR)
iv.Diencephalic seizure (Paroxysmal HTN, intense
diaphoresis, flushed skin and mydriasis)
 Disturbance of body weight
Hypothalamic obesity : Lesion in
 Ventromedial hypothalamus
 Paraventricular nucleus
 Uncontrolled voracious
appetite causing the pt.
extreme obesity
 Disturbance of body weight(contd..)
Hypothalamic weight loss: Lesion in
 Lateral hypothalamus
 Dorsomedial nucleus
 May be associated with
Hypothalamic tumour
or disorder of behavior
(Anorexia Nervosa)
 Adolescent girls are affected
 Developmental disorders

Midline cleft syndromes:

Developmental abnormalities of Skull and brain
with Hypopituitarism (varying degree)
 Developmental disorders (cont..)

Septo-optic dysplasia:
• Agenesis of septum pellucidum
• Hypoplastic optic nerve
• Hypothalamic precocious puberty
• Coronal T2 MRI showing absent septum pellucidum with
characteristic configuration of frontal horns. Also note very
thin optic chiasm (arrows). Thin corpus callosum and low
fornices. Optic nerves are hypoplastic (not shown). Pituitary
has normal appearance in this case. This is a case of
Septooptic Dysplasia that is also known as De Morsier
Syndrome.
 Developmental disorders (cont.)
• Kallman’s syndrome:
 X-linked recessive
 Clinical features
Hypogonadism
Anosmia
Tall stature
Mental retardation,Deafness, colour blindness
Absent secondary sexual characteristics
 Developmental disorders (cont.)
 Investigation
 Low GnRH, LH, FSH, testosterone
 MRI : Hypoplastic or absent olfactory bulbs

T2 MRI through frontal lobe showing absent olfactory bulb

 Developmental disorders (cont.)

Coronal T2 MRI through ant. fossa showing absent olfactory bulb

and hypoplastic left olfactory sulcus
 Pituitary-hypothalamic glioma

Usually compresses the optic chiasma and

causes visual impairment
 Hamartoma

Gelastic seizure
is caused by a
hamartoma of
the hypothalamus
Hamartoma
 Craniopharyngioma
Origin : Remnants of Rathke’s pouch
Types :
 Adamantinoma (Children)
 Papillary (Adult)
Presentation:
 Raised ICP
 Visual field defect
 Hypopituitarism, commonly DI
Craniopharyngioma (contd)
Invasive craniopharyngioma: A large complex
solid and cystic sellar-suprasellar mass lesion
compressing optic chiasma and hypothalamus,
and extending upward into the third ventricle.
A 53 year old man with memory and behavior disturbance
 Neurosarcoidosis
Neurosarcoidosis occurs in approximately 5%
cases of all sarcoidosis.

Presentation:
• Aseptic meningitis
• Cranial nerve palsy
• Hypothalamic dysfunction : DI
• Tumor like effect: seizure, hemiparesis
 Neurosarcoidosis (cont.)
Investigations
 Chest X-ray
 Histopathology/cytology
 Serum calcium
 Serum ACE
 CSF study (including ACE level)
 MRI brain
Leptomeningeal involvement
 Neurosarcoidosis

• Around pituitary stalk Parenchymal involvement

 Neurosarcoidosis (cont.)

Treatment:
• Corticosteroid
• Cyclosporin
• Cyclophsphamide
• Radiation of focal lesion
Langerhans cell histiocytosis (LCH) in a young patient with systemic
LCH: Coronal FLAIR (a) image shows thickening of pituitary stalk and
optic chiasma (thin white arrow) and a granulomatous mass lesion sitting
on the top of optic chiasma (thin black arrow). None to minimal
enhancement of the lesion is seen on T1W postcontrast image (b) Note,
associated obstructive hydrocephalus.
Lymphocytic hypophysitis in a 16-year-old girl with diabetes insipidus and growth hormone
deficiency: Postcontrast T1W coronal (a) and sagittal (b) images show slight enlargement of
pituitary gland and thickening of the infundibulum with minimal enhancement (arrow). The
fossa is not enlarged. Also note, absence of posterior bright spot.
Suprasellar germinoma: Coronal T2W FLAIR (a) and
postcontrast T1W (b) images show a solid,
heterogeneous, moderately enhancing suprasellar mass in
hypothalamic/infundibular region. Multiple
nonenhancing, hypointense, intratumoral areas are present
representing intratumoral hemorrhage or calcification.
Postsurgical biopsy proved suprasellar germinoma .
Tubercular hypophysitis: Postcontrast T1-weighted coronal images show enlarged pituitary gland with
intraglandular ring enhancing tuberculoma (thin black arrow), and thick enhancing pituitary stalk (thin
white arrow). Thickening and enhancement of diaphragma sellae (thick white arrow) is also noted.
Radiographic variant of Wernicke-Korsakoff syndrome