1. 1. HYPOTHALAMUS AND ITS CONNECTIONS (APPLIED) Dr. ATM HASIBUL HASAN MD (Neurology) Thesis Part Student Dhaka Medical College
2. 2. Who should deal? Endocrinologist Neurologist
3. 3. Alexis
4. 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. 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. 6. 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
7. 7. 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
8. 8. MRI
9. 9. a. Optic chiasm b. Median eminence c. Mamillary bodies
10. 10. MTI
11. 11. fMRI
12. 12. 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
13. 13. Diseases Developmental disorders Midline cleft defects Kallmans syndrome Septo-optic dysplasia Infiltrative and inflammatory diseases Sarcoidosis Tuberculosis Langerhans cell histiocytosis Encephalitis
14. 14. Cont Space occupying lesions Craniopharyngioma Glioma Meningioma Hemangioblastoma Hamartoma Cysts : Epidermoid, dermoid, colloid Metastasis Lymphoma and leukaemia
15. 15. Cont. Vascular Stroke Arterio-venous malformation Aneurysm Functional abnormalities Isolated hormone or receptor defect Systemic illness and psychosocial deprivation
16. 16. Cont Iatrogenic Parasellar surgery Cranial irradiation Miscelleneous Hydrocephalus Pituitary adenoma Wernickes encephalopathy
17. 17. Effects Dysfunction Endocrine Autonomic regulation Temperature regulation Eating behavior Mechanical effects Optic chiasma compression Hydrocephalus
18. 18. Endocrine dysfunctions Hypofunctions Growth hormone deficiency Hypothalamic hypogonadism Secondary hypothyroidism Central diabetes insipidus Pituitary insufficiency
19. 19. Cont. Hyperfunctions Hypothalamic precocious puberty Hypothalamic hyperprolactinaemia Syndrome of inappropriate ADH secretion
20. 20. Abnormalities of Growth: (Def/Excess of GHRH/GH) Dwarfism Acromegaly Gigantism
21. 21. Cont. Causes of GH Deficiency: Idiopathic / functional Congenital idiopathic growth hormone deficiency Emotional deprivation syndrome GH deficiency in chronic illness Hypothalamic gangliocytomas Pituitary tumor * GH deficiency due to hypothalamic disorder are mostly functional
22. 22. Cont. Hypothalamic hypogonadism Causes Isolated GnRH deficiency Structural lesion of hypothalamus Hyperprolactinaemia
23. 23. 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
24. 24. 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)
25. 25. Precocious Puberty Abnormally early onset of androgen secretion in boys ( 50% improvement after administration of DDAVP RIA of plasma ADH: usually 20 mEq/L Urine osmolality > serum osmolality Often similar clinical context
26. 37. Distinguishing features between SIADH & CSWS
27. 38. Disturbance of temperature regulation Hyperthermia: Anterior hypothalamic lesion (Preoptic) Hypothermia : Posterior hypothalamic lesion Persistent temparature of 35C (95F) Poikilothermia: Large lesion in the posterior hypothalamus
28. 39. Disturbance of temperature regulation Causes: Structural stroke, head injury, surgery, demyelination Functional Spontaneous recurrent hypothermia o Shapiros syndrome (cholesteatoma of 3rd V.) o Reverse Shapiros syndrome
29. 40. 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
30. 41. 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)
31. 42. Disturbance of body weight Hypothalamic obesity : Lesion in Ventromedial hypothalamus Paraventricular nucleus Effect- Uncontrolled voracious appetite causing the pt extreme obesity
32. 43. 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
33. 44. 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
34. 45. Developmental disorders Midline cleft syndromes: Developmental abnormalities of Skull and brain with Hypopituitarism (varying degree)
35. 46. Cont. Septo-optic dysplasia: Agenesis of septum pellucidum Hypoplastic optic nerve Hypothalamic precocious puberty
36. 47. 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
37. 48. Kallmans syndrome: X-linked recessive Clinical features Hypogonadism Anosmia Tall stature Mental retardation, Deafness, colour blindness Absent secondary sexual characteristics Kallmans syndrome Normal
38. 49. Developmental disorders (cont.) Investigation Low GnRH, LH, FSH, testosterone MRI : Hypoplastic or absent olfactory bulbs MRI through frontal lobe showing absent olfactory bulb
39. 50. Rathke Cleft Cyst Benign sellar cyst Derived from Rathke Pouch remnant Presents in 5th-6th decade Features of compression of pituitary or hypothalamus
40. 51. Craniopharyngioma Origin : Remnants of Rathkes pouch (Craniopharyngeal duct) Types : Adamantinoma (Children) Papillary (Adult) Presentation: Raised ICP Visual field defect Hypopituitarism, commonly DI
41. 52. 8 yr old boy - Adamantinoma 39 yr old man Papillary CP
42. 53. 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
43. 54. Haemangioblastoma are benign vascular tumor (2%) Usually 35-45 yrs of age Rarely affects hypothalamus- mostly Von Hippel Lindau Hemangioblastoma
44. 55. Encephalitis Mostly viral Presents with fever, Central DI, SIADH
45. 56. 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
46. 57. Neurosarcoidosis Neurosarcoidosis occurs in approximately 5% cases of all sarcoidosis. Presentation: Aseptic meningitis Cranial nerve palsy Hypothalamic dysfunction : DI Tumor like effect: seizure, hemiparesis
47. 58. Neurosarcoidosis (cont.) Investigations Chest X-ray Histopathology/cytology Serum calcium Serum ACE CSF study (including ACE level) MRI brain
48. 59. Neurosarcoidosis (cont.) Treatment: Corticosteroid Cyclosporin Cyclophsphamide Radiation of focal lesion
49. 60. Hypothalamus Love Living Your For