PROF.DR.K.H.NOORUL AMEEN’S UNIT M6 DR.RAKESH PINNINTI

An Interesting case of Paraplegia

Chief complaints

Back ache 1month Swelling in the right cheek 15days Hoarseness of voice 15days Reduced sensation in lower limbs 10days Inability to use lower limbs 8days Incontinence of urine & feces 5days Difficulty in lifting hands above shoulders 3days Deviation of angle of mouth to L side 2days

History of presentation

Patient a 29yr old male recently diagnosed as HBsAg positive, has had complaints of back ache from past 1 month, insidious persistent, dull aching quality, pain remitted occasionally but was particularly severe during nights when he laid on the bed

Pain gradually increased in severity & was disturbing his daily routine from past 20 days, pain radiated down along the thighs & such radiation of pain aggravated on activities,

h/o swelling in the right cheek from past 10 days , insidious in appearance but increasing in size, swelling was never painful, not associated with difficulty in opening mouth, or was painful during eating food.

Patient initially had complaints of clumsiness during walking, with difficulty standing from seated position, patient had such problems since 20-30 days but from past 10 days he progressively felt increasing difficulty in performing his daily routine

Patient was bed ridden with only flicker of movements in RLL, at presentation.

Patient had hoarseness of voice from past 15days, sudden onset persistent no aggravating or relieving factors associated with occasional difficulty in breathing, & noisy respiration (stridor).

H/o band like sensation around umbilicus. No h/o fever/cough/expectoration No h/o chest pain/palpitations/dyspnea No h/o headache/LOC/syncope/seizures/alter

sensorium No h/o wasting or fasciculations No h/o involuntary movements

Past history : patient recently diagnosed with Hepatitis B 6 months back & was not on any treatment for it, disease activity not known at the presentation.

no H/O TB/DM/SHT/ Personal history: occ. smoker, alcoholic. Family history: no h/o any relevant illness

GENERAL EXAMINATION

Pt conscious, oriented, not anemic not jaundiced, no cyanosis, no clubbing.

Pulse 86/mt,BP102/66 mmHG CVS&RS normal Lymphnode examinationR upper cervical multiple(4-6), matted non tender, firm in

consistency, 3*4cm in size with normal overlying skin.R supra clavicular lymphnodes 3-4 L lower deep cervical, posterior group, supraclavicular R & L axillary (medial&apical)R & L inguinal group(horz)Thyroid enlarged, firm consistency non tender, smooth

surfaceParotid R enlarged firm consistency non tender, lobulated

surface

Patient picture 1

Patient picture 2

CNS EXAMINATION

HIGHER MENTAL FUNCTIONS

Patient was conscious, oriented in time, place & person, MMSE >25 no cognitive impairment (short orientation memory conc test)

Speech – Comprehension-good Hoarseness +

CNS Examination

CRANIAL NERVES

R LMN FACIAL PALSY R LMN GLOSSOPHARYNGEAL PALSY R VOCAL CORD PALSY (10TH N)

Rest of cranial nerves normal

MOTOR SYSTEM

R L TONE: UL NORMAL NORMAL LL FLACCID FLACCID BULK: NORMAL NORMALPOWER UL 4/5 4/5 LL 1/5 0/5REFLEXES biceps R R Triceps R R Ankle ABSENT ABSENT Knee ABSENT ABSENT PLANTAR B/L NO RESPONSE

ALL SUPERFICIAL REFLEXES ABSENT( ABD;CREMAS;SP)

IMPRESSION: FEATURES SUGGESTIVE OF PARAPLEGIA

CNS Examination

SENSORY

Patient had normal perception of all sensation upto the level 1 cm above umbilicus

At & below the level of umbilicus upto pubic symphysis reduced sensibility to to pain vibration temp.

Below pubic sym, & lower limbs including the saddle region absent perception of vibration, pain, temp, fine touch.

Investigations

HB 10.8; TC 8000; DC P62 L38; PLATELET 1.8; PCV 31; MCV 78.5 MCH 26.0 MCHC 35

RFT normal (RBS 67; Creat 0.8 Urea 26) LFT normal (TB 1.0 SGOT 42 PT 38 ALP 126) T3 114ng/dl; T4 6.02m/dl; TSH 6.781mIU/ml HIV I II Negative HBsAG POSITIVE HCV Negative Serum uric acid : 4.3ng/dl

USG abdomen & pelvis Mild hepatomegaly, fatty liver, minimal ascites,

pericardial & pleural effusion, umbilical hernia. ChestXray : WNL ECG : Sinus tachycardia MRI SPINE

CT THORAX

CT ABDOMEN

MRI SPINE

C

Hematology

Peripheral Smear Normocytic normochromic aneamia

Bone Marrow AspirationNormocellular smearMyeloid:erythroid ratio 10:1Myeloid series normal in maturation & morphologyMegaryocytic & erythroid series Normal

• Serum LDH 760/dl

Histopathology

FNAC of the cervical lymphnode Lymphoproliferative disorder ? Hodgkins lymphoma

Excision Biopsy of SupraClavicular LN Features suggestive of Anaplastic Large cell Lymphoma, suggested immunohistochemistry with ALK for further evaluation

Excision Biopsy of Cervical LN (Apollo) High grade malignant neoplasm with features suggestive of Large Cell

Lymphoma

Histopathology pic 1

Histopathology pic 2

Expert opinions sort

Oncology ENT Neurosurgery Radiotherapy General surgery Medical GE

DIAGNOSIS

Generalized lymphadenopathy. Compressive myelopathy. HBsAG positive.

ANAPLASTIC LARGE CELL LYMPHOMA (IVB) METASTIC EPIDURAL SPINAL CORD COMPRESSION SYNDROME

(MESCC)

Discussion topics

Clinical features and diagnosis of Neoplastic epidural spinal cord compression,

Treatment and prognosis of Neoplastic epidural spinal cord compression

MESCC

Metastatic epidural spinal cord compression (MESCC) is defined as radiographic evidence of an epidural metastatic lesion that is causing displacement of the spinal cord from its normal position in the spinal canal.

This condition occurs in roughly 5% to 10% of cancer patients and in up to 40% of patients who have concurrent nonspinal bone metastases

Vertebral metastases are far more common than is ESCC. Roughly 30% of patients with cancer develop

symptomatic spinal metastases during the course of their illness, and up to 90% of cancer patients have metastatic lesions within the spine at the time of death.

Approximately 20 percent of cases of ESCC are the initial manifestation of malignancy

The highest incidence of clinically detected spinal metastases occurs during midlife (40 to 65 years of age), which corresponds to the period of increased cancer risk.

Primary breast, lung, and prostate represent the most common histologies metastatic to spine, reflecting both their higher prevalences and their tendencies to metastasize to bone, each of which accounts for about 20 percent of cases

Renal cell carcinoma, non-Hodgkin lymphoma, and plasmacytoma or multiple myeloma are other frequent causes of ESCC

Locations of metastatic lesions of the spine

Localization within the spine

ESCC most commonly arises in the thoracic spine. Approximately 60 percent of cases occur in the thoracic spine, 30 percent in the lumbosacral spine, and 10 percent in the cervical spine

Some studies have suggested that certain types of tumors have a tendency to metastasize and produce ESCC within specific spinal regions, such as lung cancer in the thoracic spine, and renal, prostate and gastrointestinal cancers in the lower thoracic and lumbar spine

CLINICAL EVALUATION

Back Pain Three classic pain syndromes affect patients with Spinal

metastases: local, mechanical,& radicular .Local pain is usually described by patients as a persistent

“gnawing” or “aching” pain emanating from the region of the spine that is affected by metastatic disease.

Mechanical pain, also known as axial back pain, is aggravated by movement, activity, or simply increasing weight-bearing forces on the spinal segment affected.

Radicular pain may occur when spinal lesions compress or irritate an exiting nerve root, yielding pain in the dermatomal distribution of the involved root that is often described as “sharp,” “shooting,” or “stabbing.

Motor and Autonomic Dysfunction

The second most common presenting complaint of patients with vertebral metastases is motor dysfunction, which can manifest as myelopathy and/or radiculopathy.

Bladder dysfunction is the most common autonomic finding and commonly correlates with the degree of motor dysfunction.

Severe autonomic dysfunction with urinary retention, constipation, and loss of control of bowel or bladder function is a late and particularly ominous finding because full paraplegia can follow within hours.

Neurologic status at the time of diagnosis, particularly motor function, has been shown to correlate with prognosis from MESCC, thus reinforcing the concept that diagnosis prior to the development of a neurologic deficit is of paramount importance

For this reason, new-onset back or neck pain in a patient with known cancer must be considered spinal metastatic disease until proven otherwise.

Sensory findings

Sensory findings are less common than motor findings but are still present in a majority of patients at diagnosis.

Patients frequently report ascending numbness and paresthesias

When a spinal sensory level is present, it is typically one to five levels below the actual level of cord compression.

Lhermitte's phenomenon, the experience of electricity down the spine with neck flexion, may be seen in multiple sclerosis, cervical spondylotic myelopathy, cisplatin-induced neurotoxicity, radiation-induced myelopathy, neck trauma and rarely with an epidural or subdural neoplasm.

Flowchart for initial workup of patient with suspected spinal metastatic disease

The median survival for patients ambulatory prior to RT is eight to ten months compared to two to four months for those who are nonambulatory. For those who remain nonambulatory at the conclusion of RT, the median survival is only one month

DIFFERENTIAL DIAGNOSIS

Musculoskeletal disease Spinal epidural abscess Metastatic disease Vertebral metastases without epidural extension Intramedullary metastases Leptomeningeal metastases Malignant plexopathy

• Radiation myelopathy

• Other : Spinal epidural cavernous hemangiomas ; Spontaneous nontraumatic spinal epidural hematomas, Meningiomas and neurofibromas ; extramedullary hematopoiesis due to thalassemia or chronic myeloproliferative or myelodysplastic disorders ,epidural involvement by rheumatoid arthritis, sarcoidosis, or tophaceous gout

Flowchart for management of spinal metastatic disease

TREATMENT

Hormonal Therapy, Chemotherapy, and Medical Therapy Corticosteroids Bisphosphonates Analgesia Conventional Radiation Therapy Surgical Approaches and Techniques Biomechanical Considerations Spinal Stereotactic Radiosurgery Percutaneous Vertebroplasty and Kyphoplasty

Hepatitis B virus infection and risk of non-Hodgkin lymphoma in South Korea: a cohort study

HBsAg, chronic Lymphoproliferative disorders, and cirrhosis of liver

Reactivation of hepatitis B virus infection with persistently negative HBsAg on three HBsAg assays in a lymphoma patient undergoing chemotherapy (February 2010)

T h a n k y o u