Sper-ittzem Specimen2 2 contained a large incidental fluidcontaining cyst measuring 7 x 5 x 3 cm that markedly displaced the left anterior temporal cortex (Fig 3). The patient was a 59-year-old woman who had died of widespread metastases of breast cancer, but she had had no known neurological disorder, and subsequent examination showed no metastatic tumor. T h e whole brain weighed 1 , 4 5 0 gm, the forebrain 1,270 gm, and the brainstem and cerebellum 175 gm. Each cerebral hemisphere weighed 630 gm and had a volume of 6 2 5 cc. The results are straightforward and clear-cut. In spite of the absence grossly of the anterior temporal lobe and the deformity of the frontotemporal cortex adjacent to the cyst, there was n o significant difference in the weight or volume of the two cerebral hemispheres to suggest any evidence of agenesis o r hypoplasia involving the affected cerebral hemisphere. Supported in part by Mental Retardation and Child Development Research Grant 5P0l HDO2274 from the National Institute of Child Health and Human Developinent.

References 1. Robinson RG: lntracranial collection of fluid with local bulging of the skull. J Neurosurg 12345-351, 1955 2. Robinson RG: Local bulging of the skull and external hydrocephalus d u e to cerebral agenesis. Br J Ra&~l 31:691-700, 1958 3. Robinson RG: The temporal lobe agenesis syndrome. Brain 87:87-105, 1964 4. Shaw CM, Alvord EC Jr: “Congenital arachnoid” cysts and their differential diagnosis, in Vinken PJ, Bruyn G W (eds): HanJhook of Clinical Neurology. Amsterdam, North-Holland, 1977, VOI 31, pp 7 5 - 1 3 > 5. Starkman SP, Brown TC, Linell EA: Cerebral arachnoid cysts. J Neuropathol Exp Neurol 17:484-500, 1958

Progressive Myelopathy Due to Extramedullary Hematopoiesis: Case Report and Review of the Literature M. Stahl, MD, PhD, G a r y Ellinger, MD, Richard Baringer, MD

Stephen J,

Extramedullary hematopoiesis (EMH) in the spinal epidural space is a rare but treatable cause of progressive paraparesis in patients with a variety of hematological and systemic disorders. We report a case of extensive thoracic epidural EMH causing progressive spastic paraparesis in a patient with agnogenic myeloid metaplasia. The literature describes 1 1 additional cases of myelopathy due to epidural EMH. All patients had EMH in the midthoracic region with elevated cerebrospinal fluid protein and abnormal spine roentgenograms or myelograms. Treatment with decompressive laminectomy, radiation therapy, or both, resulted in marked or complete recovery in 7 of 10 patients. Patients who develop paraparesis and who have a disorder associated with EMH should be evaluated rapidly for this treatable condition. Stahl S M , Ellinger G, Baringer JR: Progressive m y r l o p a t h y d u e t o extramedullary hematopoiesis: case report a n d review o f t h e literature. A n n

Neurol 5:485-489, 1979

Extramedullary hematopoiesis (EMH) accompanies a number of hematological and chronic medical diseases [26]. Hernatopoietic activity is usually induced outside the bone marrow by failure of normal marrow to produce blood cells. It occurs most commonly in the spleen, liver, and lymph nodes but may also arise in unusual sites such as breast [ 3 , 91, peripheral nerve, and epididymis [9]. Sporadic case reports over the past twenty years have also documented that dura mater, especially in the region of the thoracic spinal cord, may be a site of E M H [ 1, 4-6, 11, 15, 18, 19, 2 2 , 251. I t has not been widely appreciated that such hematopoietic activity can act as a mass lesion to produce progressive paraparesis. H e r e we report a patient in whom this phenomenon occurred, and we From the Neurology and Pathology Services, Veterans Administration Hospital, and the Department of Neurology, University of California, San Francisco, CA. Accepted for publication Sepr 29, 1978. Address reprint requests to D r Baringer, Neurology Service, Veterans Administration Hospital, 4150 Clement St, San Francisco, CA 94121.

0364-5134/791050485-05$01.25 (9 1978 by S t e p h e n M. Stahl

485

review the clinical features and response to treatment in 11 other cases of progressive myelopathy due to EMH in the world’s literature. A 54-year-old man had developed agnogenic myeloid metaplasia at age 47. A splenectomy at age 49 and subsequent androgen treatment both failed to improve his anemia, which had required transfusion of more than 100 units of blood over seven years. At age 50, the patient developed lower extremity weakness. H e had normal strength, tone, and reflexes in the upper extremities with mildly increased tone, symmetrically hyperactive reflexes with bilateral extensor plantar responses, and mild distal, symmetrical weakness of the lower extremities. A rnyelogram showed narrow anteroposterior diameter of the entire spinal canal with minimal bony encroachment at C5-6. Cerebrospinal fluid protein was 53 mg per deciliter. A bone scan was normal, an indium marrow scan showed no uptake in the spine, and a technetium-colloid scan showed no marrow hematopoietic activity. T h e paraparesis was stable over the next three years, and the patient continued to require frequent transfusions. When he was 53, the paraparesis progressed to the point that he could not walk; he became incontinent, and was confined to a wheelchair. His strength was normal but skeletal muscle tone was mildly increased and reflexes were symmetrically hyperactive in the upper extremities; he had diffuse spastic weakness, marked hyperreflexia, sustained ankle clonus, and bilateral extensor plantar responses in the lower ex-

486 Annals of Neurology Vol 5 No 5 May 1079

tremities. Sensation was intact except for mild vibratory loss in the toes. A myelogram showed narrowing at C5-6 with a complete block at L3. CSF protein was 67 mg per deciliter. A technetium-colloid scan showed abnormal cellular marrow in the lumbar region and sacrum. Because of intercurrent medical problems (sepsis and congestive heart failure), h e was not considered to be a surgical candidate. Nor was he considered for spinal irradiation because of progressively increasing transfusion requirements and because the only functional hematopoietic tissue was confined to the spinal axis. The patient died six months later of spontaneous pneumothorax and lactic acidosis.

Autopsy Findings At autopsy, marked hepatomegaly (4,800 gm) with mixed nodular cirrhosis, generalized lymphadenopathy, and hemosiderosis were found. EMH occurred diffusely in hepatic and lymph nodal sinusoids, accessory spleen, and testes. In addition, large aggregates composed entirely of hematopoietic tissue were present in liver, renal pelves, and within the lumbar and thoracic spinal canal. T h e deposits of EMH were exclusively epidural; they extended from C3 to L5 and compressed the adjacent spinal cord, producing degeneration and gliosis of posterior and lateral columns (Figs 1, 2 ) .

Fig I . EMIt i.c prrsent as an epidzirui ma.r.5 udjacent t o the spinal cord. IH&E, xI(1.1

F i g 2.High-pou er 2 leu' of epidural mau shouir normobkusts. niegakaryocytes, und immature myeiotd celh. ( H & E , x400.i

Discussion Eleven cases of spinal cord compression due to E M H have been reported [ I , 4-6, 11, 15, 18, 19, 22, 251; our patient is the twelfth. The patients were mostly men (10 of 12), aged from 17 to 60 years, with one of three associated diseases: thalassemia (in 6), myelofibrosis (in 5), and transposition of the great vessels (in 1). Detailed clinical descriptions are not available for all patients, but all 12 had some degree of weakness in the legs, and 8 of 9 patients for whom adequate information is available had diminished appreciation of primary sensory modalities (vibratory and position sense were diminished more than light touch and pain). Only 2 patients had urinary or fecal incontinence and only 3 had any back pain at the time of diagnosis. Reflexes were increased, decreased, or normal. The mass of hematopoietic tissue was invariably located in the midthoracic region and usually extended over several spinal levels (Table). The CSF

protein was elevated in those patients for whom information is available. The diagnosis of spinal cord mass was made in 10 of 12 cases by myelography; in the remaining 2, abnormalities were seen in the plain spinal roentgenograms. Treatment with decompressive laminectomy, radiation therapy, o r both, was undertaken in 10 of the 12 patients; 7 of 10 recovered neurological function substantially o r completely, 2 showed little or no improvement, and 1 died postoperatively (see the Table). Thus, the onset of progressive paraparesis in a patient with thalassemia or myelofibrosis should suggest the presence of a thoracic epidural mass of EMH tissue. Investigation of these patients should include evaluation of CSF protein, plain films of the thoracic spine, and myelography, all of which would be expected to reveal abnormalities in patients with EMH. Furthermore, treatment with surgery, irradiation, o r both, would be expected to yield a high percentage of excellent results. The list that follows includes many of the diseases with which E M H has been associated. Associated diseases in cases of EMH with myelopathy 11, 4-6, 11, 15, 18, 19, 22, 251: chalassemia (6 patients),

Case Report: Stahl et al: Progressive Myelopathy

487

Chuructertctii r oJ Putieiiti

ii

ith ProKvrssri e illyeloputby Due Patient’s Age (yr) and Sex

Source

MIhind ukalasuriyL et al, 1977 1221

to

S p l d Epidural Extrattiediillury HematopotesiJ

Disease H e m o g l o b i n E/ t h alas sem i a Transposition of g r e a t vessels

24, F

141

17, M

Bree et al, 1974 L41

37,M

Myelofibrosis

Cauthen e t al, 1968 151 (and Sorscidhl e t al.

40, M

Thalassemia

Cauthen et al, 1968 L5J

47, M

Thalassemia

Close e t al, 1958 [hl

60, M

Myelofibrosis

B r e e et al. 1 9 7 4

1%

t

1.251,

Symptoms and Duration

Locat ion of Mass

18 m o : progressive

T4-9

paraparesis 1 mo: progressive paraparesis Duration unknown: paraparesis 2 mo: progressive paraparesis and urinary retention 2 mu: progressive paraparesis

8 m o : progressive

T8-9 T3-5, L1-2 T9- 10 T4-8

T5-12

paraparesis Appleby e t al, 1964 I11

42, M

Thalassemia

21, M

Thalassemia

G a t t o e t al. 1954 11 11

26, M

Thalassemia

Lowman et al, 1963 I 181

31, M

Myeloh brosis

Luyendijk e t al, 1‘975

3 mo: progressive

Myelohbrosis

58, F

paraparesis Duration unknown: progressive paraparesis 12 mo: back pain

1191 H e f t n e r and Koehl,

Tll-12

T5-8 T8-L3

1970 1151

2 m o : progressive parapare s i s 12 mo: progressive

T10

T7-9

paraparesis Stahl e t al (this r e p o r t )

4 yr: progressive

Myelofi brosis

58, M

C3-L5

paraparesis PhIN

=

polyniorptionuclcar leukocyte.

myeloilbrosis ( 5 patients), and transposition of great vessels ( 1 patient). Associate~ldiseases in cases o f thoracic EblH without myclopathy [ 2 , 3, 7, 8, 10, 12-11, 16, 17, 20, 21, 23, 211: heredirary spherocytosis ( 8 patients), thalassemia ( 3 patients), bronchopneumonia ( 2 patients), eryrhroblastosis (2 patients), osteitis fibrosis cystica ( 1 patient), and carcinoma of stomach ( 1 patient). Diseases associated with EMH (any site), 1271: s p h r rocytosis, thalassemia, pernicious anemia, myelofibrosis, erythroblastosis fetalis, kala azar, Hodgkin’s disease, lymphoma, leukemia, metastatic carcinoma, chronic infections, hyperrholesterolemia, rickets, hy pcrparathyroidism, osteitis hbrosa cystica, g o u t , congenital hemolytic anemia, and congenital heart disease.

Several of these conditions have included thoracic masses in patients without clinical evidence of myelopathy. O n e might expect that patients with the conditions appearing in the list are at risk for the development of spinal cord compression due to EMH, particularly with prolonged survival due to improved treatment of the underlying disorder.

488

Annals o f Neurology

Vol 5

No 5

May 1 9 7 9

References 1. Appleby A, Batson GA, Lassman LP, et al: Spinal cord compression by extramedullary haematopoiesis in myelosclerosis. J Neurol Neurosurg Psychiatry 27:313-316, 1964 2. Ask-Upmark E: Tumor-simulating intrathoracic heterotopia of bone marrow. Acta Radiol 26:425-440, 1945 3. Brannan D: Extramedullary hematopoiesis in anemia. Bull Johns Hopkins Hosp 41:104-136, 1927 4. Bree RL, Neiman HL, Hodak JA, et al: Extramedullary hematopoiesis in the spinal epidural space. J Can Assoc Radiol 25:297-299, 1974 5. Cauthen JC, McLaurin LP, Foster MT, et al: Spinal cord compression secondary to extramedullary hematopoiesis in two brothers. J Neurosurg 29:529-531, 1968 6. Close AS, Taira Y , Cleveland DA: Spinal cord compression due to extramedullary hematopoiesis. Ann Intern Med 48:421-427, 1958 7. Covery GW: Erythroblastosis: report of case presenting erythroblastic tumor in thoracic cavity. Am J Pathol 11:551-559, 1935 8. Dawson L: Hume lectures on haemolytic icterus. Br Med J 11963-966, 1931 9. Dieterich H: Studien uber extramedullare Blutbildung bei chirurgischen Erkrankungen. Arch Klin Chir 134:166-175, 1925 10. Foster JBT: Primary thoracic myelolipoma. Arch Pathol 65:295-297, 1958

CSF Findings

Radiological Findings

Treatment

1 PMN, protein 65

C o m p l e t e block T9

Laminectomy

C o m p l e t e recovcry

Protein 355

Coniplete block

T9

Laminectomy

Postop death

Compression TI-5, complete block L1

Irradiation

C o m p l e t e recovery

2 x 2 cm soft tissue mass T9- 10

Laminectomy

Markedly improved

Paraspinal intrathoracic masses T9-10; complete block T7 C o m p l e t e block T8

Larninectoiny plus irradiation

Markedly improved

Laminectomy plus irradiation Laminectomy plus irradia-

No improvement

2

Protein 184 Protein

235

C o m p l e t e block T12

._

Outcome

Markedly improved

tion

1 m o n o , protein 40

C o m p l e t e block T5-8

20 lymphs, protein 70

N o r m a l myelogram; rarefaction of lumbar vertebrae C o m p l e t e block T I 0

i

I

C o m p l e t e block T9

Protein 6 7

Compression C5, complete block L3

11. Gatto I, Terana V, Biondi L: Compressione sul midollo spinale da proliferazione di midollo osseo spazio epidurale in sogetto affetto da malattia di Cooley splenectomizatto. Haematologica 38:61-76, 1954 12. Gleave H H : Paravertebral heterotopia of bone marrow in a case of acholuric jaundice. J Pathol Bacteriol 42:538-539, 1936 13. Hanford RB, Schneider GF, MacCarthy JD: Massive thoracic extramedullary hematopoiesis. N Engl J Med 263: 120-123, 1960 14. Hartfall SJ. Stewart MJ: Massive paravertebral heteroropia of bone marrow in a case of acholuric jaundice. J Pathol Bacteriol 37:455-459, 1933 15. Heffner RR, Koehl RH: Hemopoiesis in the spinal epidural space. J Neurosurg 32:485-490, 1970 16. Knoblich R: Extramedullary hematopoiesis presenting as intrathoracic tumors. Cancer 13:462-468, 1960 17. Lirwer H: Myelolipoma of the mediastinum. Radiology 74: 471- 4 7 3 , I960 18. Lowman RM, Bloor CM, Newcomb AW: Roentgen manifestations of thoracic extramedullary hemaropoiesis. Dis Chest 44:154-162, 1963

Laminectomy plus irradiation None

Markedly improved Autopsy cltagnosis

Laminectorny plus irradiation

Markedly iniprovecl

Laminectomy plus irradiation None

Slightly improved P r o g r e w v e cfeterioration

19. Luyendijk W, Went L, Schaad HDG: Spinal cord compression due to extramedullary hematopoiesis in homozygous thalassemia. J Neurosurg 42:212-216, 1975 20. Malamos B, Papavisiliou C, Avramidis A: Tumor simulating intrarhoracic extramedullary hemaropoiesis. Acra Radio1 57:227-2 3 1, 1962 21. Marinozzi V: Aspetti insoliti dell'iperplasia midollare nelle anemie emolitiche. Haematologica 43:737-759, 1958 22. Mihindukalasuriyz JCL, Chanmugan D, Machado V, et al: A case of paraparesis due to extramedullary hematopoiesis in HbE-thalassaemia. Postgrad Med J 53: 393--397, 1977 23. Plonskier M: Uber tumorformige (extramedullare, heterotope) subpleurale Knochenmarksherde. Virchuws Arch Pathol Anat 277:804-809, 1930 24. Saleeby ER: Hererotopia of bone marrow without apparent cause. Am J Pathol 1:69-76, 1925 25. Sorsdahl OS, Taylor PE, Noyes WD: Extramedullary hematopoiesis, mediastinal masses, and spinal cord compression. JAMA 189:343-347, 1964 26. Ward H, Black M: The natural history of agnogenic myeloid metaplasia. Medicine 50:357-420, 197 1 27. Wintrobe MM: Clinical Hematology. Fifth edition. Philadelphia, Lea & Febiger, 1961

Case R e p o r t : Stahl e t al: Progressive Myelopathy

489

Progressive myelopathy due to extramedullary hematopoiesis: case report and review of the literature.

Sper-ittzem Specimen2 2 contained a large incidental fluidcontaining cyst measuring 7 x 5 x 3 cm that markedly displaced the left anterior temporal co...
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