Findings and courses of nine patients with lymphocytic hypophysitis treated by high dose methylprednisolone pulse therapy
| Age, sex | Clinical presentation | Laboratory findings | MRI | Biopsy | Follow up | |||||
|---|---|---|---|---|---|---|---|---|---|---|
| Case 1 23 y, M | Slowly progressive hypopituitarism over 6 years | Impaired pituitary functions: C, G, S, L, T | Global symmetric enlargement of anterior pituitary lobe with slight suprasellar extension and marked homogeneous contrast enhancement. Thickened, enhancing sellar diaphragm. Thickened and markedly enhancing pituitary stalk | Thickened sellar endosteum. Whitish, firm sellar content | 38 months. No improvement of pituitary function. Slight shrinkage of enlarged sellar content and pituitary stalk | |||||
| Case 2 29 y, F | Headache and chiasm compression since 4 months, starting in the last trimester of pregnancy | Impaired pituitary functions: C, G, S, L | Global symmetric enlargement of anterior pituitary lobe with suprasellar extension and marked homogeneous contrast enhancement. Thickened and markedly enhancing pituitary stalk | Thickened sellar endosteum. Whitish firm sellar content | 36 months. Normalisation of G and S pituitary function, C and L impairment unchanged. Shrinkage of sellar content nearly to an empty sella, shrinkage of thickened pituitary stalk | |||||
| Case 3 36 y, F | Oligomenorrhoea for 6 months | Impaired pituitary functions: G, S, L | Slightly asymmetric enlargement of anterior pituitary lobe with suprasellar extension and marked homogeneous contrast enhancement. Pituitary stalk of normal appearance | Reddish inflammatory nodule of firm consistence, delineable from the pituitary. Complete removal. | 24 months. Persistent impairment of G, S, L function, postoperatively persistent DI. MRI not evaluable because of extensive surgery | |||||
| Case 4 44 y, F | Sudden onset of diabetes insipidus (DI) 1 month previously | Impaired pituitary functions: S, DI | Global symmetric enlargement of anterior pituitary lobe with suprasellar extension, ring-like enhancement of pituitary. Thickened and markedly enhancing pituitary stalk | Not done | 42 months. DI ceased, persistent impairment of S function. Shrinkage of the sellar content, pituitary stalk still somewhat enlarged | |||||
| CSF: 78 lympho-monocytic cells/mm3, 28 mg/dl global protein content; virus serology negative. | ||||||||||
| Case 5 40 y, F | Progressive hypopituitarism over 6 months | Impaired pituitary functions: C, T, G, L. CSF: 48 lymphomonocytic cells/mm3, 38 mg/dl global protein content; virus serology negative. | Global symmetric enlargement of anterior pituitary lobe with slight suprasellar extension and marked homogeneous contrast enhancement. Pituitary stalk of normal appearance | Not done | 36 months. Normalisation of G and improvement of T impairment, C and L impairment unchanged. Shrinkage of sellar content | |||||
| Case 6 42 y, F | Sudden onset of DI 1.5 years previously | Impaired pituitary functions: C, S, DI. CSF: 30 lymphomonocytic cells/mm3, 31 mg/dl global protein content; virus serology negative. Thyroglobulin autoantibody concentration not risen. | Sellar content of normal extent, posterior lobe not delineable. Homogeneous marked contrast enhancement of the sellar content. Pituitary stalk slightly thickened and markedly enhancing | Not done | 27 months. Normalisation of C function. Improvement of DI and S impairment. Shrinkage of the thickened pituitary stalk | |||||
| Case 7 30y, M | Sudden onset of DI 2 months previously | Impaired pituitary functions: C, DI. CSF: 33 lymphomonocytic cells/mm3, 54 mg/dl global protein content; virus serology negative. Thyroglobulin autoantibody concentration not risen. | Slight symmetric enlargement of sellar content with prominence of the posterior lobe. Marked and homogeneous contrast enhancement of the sellar content. Pituitary stalk of normal appearence | Not done | 19 months. DI ceased, persistent slight impairment of C function. No change of sellar content | |||||
| Case 8 62 y, F | Sudden onset of DI 2 months previously | Impaired pituitary functions: C, G, S, DI. CSF: 198 lympho-monocytic cells/mm3, 56 mg/dl global protein content, virus serology negative. Thyroglobulin autoantibody concentration not risen. | Sellar content of normal extent, posterior lobe not delineable. Marked and homogeneous enhancement of sellar content. Pituitary stalk thickened and strongly enhancing | Not done | 19 months. DI ceased, G normalised, S improved, persistent impairment of C function. Shrinkage of pituitary stalk | |||||
| Case 9 62 y, F | Progredient decrease of performance over 1.5 years | Impaired pituitary functions: S. CSF: 45 lympho-monocytic cells/mm3, 75 mg/dl global protein content; virus serology negative. Thyroglobulin autoantibody concentration markedly risen (1935.6 U/ml). | Global symmetric enlargement of the anterior pituitary lobe with suprasellar extension and marked homogeneous contrast enhancement. Thickened and markedly enhancing pituitary stalk | Not done | 19 months. Persistent impairment of S function. Thyroglobulin autoantibody concentration normalised (<40 U/ml). Slight shrinkage of sellar content and of pituitary stalk | |||||
C= Corticotroph; T= thyreotroph; G= gonadotroph; S= somatotroph; L= lactotroph.