4. Contents
Imaging of Hypothalamus
Diseases of Hypothalamus
Presentation of Hypothalamic disease
o Dysfunction
Hypofunction
Hyperfunction
o Mechanical effect
Brief description of some relevant diseases
5. About Hypothalamus
• Only 0.3% of total
brain, but is essential
for life
• Lies in the centre of
the limbic system
• Controller of body
homeostasis
• Disorders may be
global or partial
6.
7. Nucleus Zone(s) Region(s) Functions
Paraventricular Periventricular, Medial Anterior,Tuberal
Fluid balance, milk let-down, parturition,
autonomic & anterior pituitary control
Preoptic Medial, Lateral Anterior
Control parasympathetic
system,thermoregulation, sexual
behavior
Anterior Medial Anterior
Control parasympathetic
sysrem,thermoregulation, sexual
behavior
Suprachiasmatic Medial Anterior Biological rhythms
Supraoptic Medial, Lateral Anterior Fluid balance, milk let-down, parturition
Dorsomedial Medial Tuberal Emotion (rage)
Ventromedial Medial Tuberal Appetite, body weight, insulin regulation
Arcuate Periventricular, Medial Tuberal Control of anterior pituitary, feeding
Posterior Medial Posterior Thermoregulation
Mammillary Medial Posterior Emotion and short-term memory
Lateral Complex Lateral Tuberal Appetite and body weight control
8.
9. Imaging of Hypothalamus
Magnetic Resonance Imaging (MRI)
Magnetization Transfer Imaging (MTI)
Diffusion Weighted Imaging (DWI)
Proton MR Spectroscopy
18 FDG PET
SPECT
Intraoperative Real Time Ultrasonography
14. When to Suspect ?
• Any feature of
Diencephalic syndrome (FTT, vomiting and emaciation-
Anterior hypothalamus)
Precocious puberty (Tuber cinereum)
Hormone deficinecy
• Features suggesting mass effect on Chiasma
• Epilepsy:
Gelastic seizure
* Destructive lesion of hypothalamus never causes precocious puberty
25. A 3yr old girl presented with regular monthly PV bleeding for last 6 months. Her
parents also noticed brief spells of abnormal laughter for last few months.
Hypothalamic Hamartoma
26. Cont.
Hypothalamic hypergonadism
Causes-
Structural lesion of hypothalamus
(Hamartoma most common)
Idiopathic
More common in females (most are idiopathic)
Males are more severely affected (most are structural
lesions)
27. Precocious Puberty
• Abnormally early onset of androgen
secretion in boys (<9yrs) and
estrogen secretion in girls (<8yrs).
• 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.
• Treatment:
GnRH analogue: Leuprolide
28. A 32yr old man presented with intermittent fever, cough, breathlessness, polyuria and
polydipsia
Cranial Diabetes Insipidus due to
Sarcoidosis
31. CDI
Investigation
Water deprivation test :
Plasma osmolality > 300 mOsm/L
Urine osmolality < 600 mOsm/L
> 50% improvement after administration of
DDAVP
RIA of plasma ADH: usually <1.0 pg/ml
(n=1.4-2.7 pg/ml)
32. A 66 yrs old man presented to ER with headache, confusion and unsteadiness. He had
H/O PTB 15 yrs back. On query the attendants also reported frequent cough with
sputum production for last 6 yrs.
SIADH due to Bronchiactasis
33. Syndrome of inappropraite ADH
secretion (SIADH)
Pathogenesis :
• Excessive ADH
• Excessive water reabsorption in collecting tubule
• Dilutional hyponatraemia
34. SIADH
Causes-
CNS disorders :Stroke, infection, injury, tumor
Pulmonary disorders : Pneumonia, TB , CA
Drugs : Carbamazepine, TCA, Antipsychotic
Ectopic production of ADH: Carcinoma, thymoma,
mesothelioma, lymphoma
Idiopathic
35. SIADH
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
36. A 42 yrs old alcoholic man presented to ER with sudden fall and unconsciousness.
The situation was further complicated by alcohol withdrawal seizure. After initial
improvement patient again developed confusion at 10th day and there was postural
drop of BP. Investigation revealed nothing but hyponatremia (Na level is 122
mmol/l).
Cerebral Salt Wasting Syndrome
37. Cerebral Salt Wasting Syndrome
• Also known as Neurogenic SWS
• Caused by –
Acute intracranial diseases,
Tumor
Post neurosurgical procedure
• Pathophysiology-
Decreased sympathetic drive
BNP and ANP
• Effect- Sodium loss rather than water retention
• Fluid restriction may be dangerous
Opposes the action of ADH in RT
and also inhibit ADH release from
hypothalamus
41. Disturbance of temperature regulation
Hyperthermia:
Anterior hypothalamic lesion (Preoptic)
Hypothermia :
Posterior hypothalamic lesion
Persistent temparature of ≤ 35°C (95°F)
Poikilothermia: Large lesion in the posterior hypothalamus
42. Disturbance of temperature regulation
Causes:
Structural – stroke, head injury, surgery,
demyelination
Functional – Spontaneous recurrent
hypothermia
o Shapiro’s syndrome (cholesteatoma of 3rd V.)
o Reverse Shapiro’s syndrome
43. Disturbance of Autonomic regulation
• Acute autonomic crises (Sympathetic storm)
• An exacerbation of stress response
Causes-
• Toxic and pharmacologic agents-
Cocaine
Phenylpropanolamine
TCA overdose
OP insectisides
• Severe head injury
• Hypertensive ICH
44. Disturbance of Autonomic regulation
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)
45. Disturbance of body weight
Hypothalamic obesity :
Lesion in
Ventromedial hypothalamus
Paraventricular nucleus
Effect-
Uncontrolled voracious
appetite causing the pt
extreme obesity
46. Disturbance of body weight
Hypothalamic weight loss:
Lesion in
Lateral hypothalamus
Dorsomedial nucleus
o May be associated with
Hypothalamic tumour or disorder of
behavior
(Anorexia Nervosa)
o Adolescent girls are affected
47. Periodic somnolence and bulimia
Known as Kleine-Levin Syndrome
• Hypothalamus is responsible, but without definite
pathologic confirmation
• Usually adolescent boys are affected
• Episodic disorder characterized by somnolence and over
eating
• Pt. sleep ≥18 hrs/day, waking only to eat and attend to toilet
needs
• They appeared dull, often confused and restless
50. MRI showing absent septum pellucidum with characteristic configuration of frontal
horns. Very thin optic chiasm. Thin corpus callosum and low fornices. Pituitary has
normal appearance.
Septo optic Dysplasia/ De Morsier Syndrome
51. Kallman’s syndrome:
X-linked recessive
Clinical features
Hypogonadism
Anosmia
Tall stature
Mental retardation,
Deafness, colour
blindness
Absent secondary sexual
characteristics Kallman’s syndrome Normal
54. Rathke Cleft Cyst
• Benign sellar cyst
• Derived from Rathke Pouch
remnant
• Presents in 5th-6th decade
• Features of compression of
pituitary or hypothalamus
55. Craniopharyngioma
Origin : Remnants of Rathke’s pouch
(Craniopharyngeal duct)
Types :
Adamantinoma (Children)
Papillary (Adult)
Presentation:
Raised ICP
Visual field defect
Hypopituitarism, commonly DI
56. 8 yr old boy - Adamantinoma 39 yr old man Papillary CP
57. Hypothalamic Chiasmatic Glioma
• About 10-15% of supratentorial
tumor in children
• Presents at 2-4 yrs of age
• Diminished visual acuity
• Short stature ( GH )
• F/H/O NF-1
• Slowly progressive with
occasional spontaneous
remission
58. • Haemangioblastoma are benign vascular tumor (2%)
• Usually 35-45 yrs of age
• Rarely affects hypothalamus- mostly Von Hippel Lindau
Hemangioblastoma
61. A 61-year-old woman developed a rapidly progressive dementia
associated with visual loss. In 7 mo she was dependent for self-care.
Coronal T2-weighted [A], axial FLAIR [B], and axial contrast-enhanced
T1-weighted images [C and D] demonstrate extensive involvement of the
hypothalamic and suprasellar regions, extending laterally towards the optic
tracts, and to the left temporal lobe. The enhancing portion of the lesion is
hypothalamic and suprasellar, and there is also a component of
enhancement in the anterior portion of the temporal lobe (arrow in D).
Neurosarcoidosis involving Hypothalamus
62. Neurosarcoidosis
Neurosarcoidosis occurs in approximately 5% cases of
all sarcoidosis.
Presentation:
• Aseptic meningitis
• Cranial nerve palsy
• Hypothalamic dysfunction : DI
• Tumor like effect: seizure, hemiparesis