Download - Elderly male with Flaccid paraparesis diagnosed as Cauda Equina Syndrome due to Multiple Myeloma
Dr. Md Rashedul Islam FCPS, MRCP(UK)
Registrar, Neurology, BIRDEM
Presenting Complaints:
A 66 years old diabetic, right handed, gentleman,
hailing from Keraniganj, Dhaka got admitted in BIRDEM
General Hospital on 12th February,15 with the
complaints of-
• Difficulty in walking for 1 month• Retention of urine for 2 days
H/O Present illness
According to the statement of the patient, he was reasonably well 1 month back. Then he developed difficulty in walking which was gradual on onset associated with low back pain. His back pain was dull aching in nature, mild in severity with radiation to left lower limb, aggravated by walking, movement, change of posture relieved by taking rest. It was not associated with fever, cough, weight loss, alternation of bowel habit, h/o trauma.
H/O Present illness
He also had complaints retention of urine which was sudden on onset associated with abdominal discomfort. He was catheterized outside BIRDEM. He didn’t have complaints of urgency, hesitancy, frequency of micturation.
H/O past illness:
Nothing contributory
Socioeconomic history:
He belongs to a middle class family
Personal history:
He is non alcoholic, non smoker
Family history:
Nothing significant
Treatment history:
Insulin
Tab. Vit B complex
General examination:
Appearance: ill looking, anxiousBuilt: average Decubitus: on choiceAnaemia ++JaundiceCyanosisOedemaDehydrationClubbingKoilonychiaLeukonychia
Absent
General examination:
Neck vein: not engorged
Thyroid: not enlarged
Lymph node: not palpable
Skin pigmentation & body hair distribution: normal
Pulse: 78 b/min
BP: 120/70 mmHg
Temp:98° F
RR: 16 breaths/min
Catheter is situ
• Higher psychic function : Conscious, Oriented• Speech: Normal• Cranial nerves : Intact• Fundus: Normal• GCS: 15/15
NERVOUS SYSTEM EXAMINATION
• Wasting of lower limb muscles globally
Muscle Rt. UL Lt. UL Rt. LL Lt. LL
Bulk Normal Normal Reduced Reduced
Tone Normal Normal Decreased Decreased
Power 5/5 5/5 4/5 3/5
Involuntary movement
Absent Absent Absent Absent
MOTOR FUNCTION:
Reflex B T S K A Abd Plantar
Right ++ ++ ++ Absent
Absen
t
Present Absent
Left ++ ++ ++ Absent
Absen
t
Present
Absent
Sensory system:Pain Temp Touch Vibratio
nPosition sense
Right upper limb
All modalities of sensation was diminished at L4/L5/S1 dermatomal distribution on left side along with saddle anaestheisa
Right lower limb
Left upper limb
Left lower limb
• Sign of Meningeal irritation - Absent
• Cerebellar sign : Absent
• Gait: Antalgic gait
Musculoskeletal System
Examination of Spine: • Tenderness on percussion at level of lower lumbar
area• Restricted spinal mobility • Straight leg raising test:
limited to 40° (left side) & 90°(Right side)
Systemic examinations
Other systemic examination was normal
A 66 years old diabetic gentleman got admitted with the complaints of progressive difficulty in walking with dull aching, mild low back pain radiating to left leg, associated with retention of urine. He had anaemia, restricated spinal mobility & tenderness on lower spine. on examination of lower limbs, he had wasting, hypotonia, diminished muscle power, areflexia, absent plantar & antalgic gait. All modalities of sensation was diminished at L4/L5/S1 dermatomal distribution on left side along with saddle anaestheisa . SLR was restricted on left side. Other systemic examination was normal.
Salient feature
Provisional diagnosis
• Diabetes Mellitus Type 2• Cauda equina syndrome due to discitis
or metastasis?
Differential diagnosis
• Conus medullaris syndrome• Traumatic peripheral nerve lesions• Acute inflammatory demyelinating
polyradiculoneuropathy
Investigations
CBC:
Hb % - 8.2
WBC -6800 cu/mm
Neu-65 %
Lymph- 30%
Mono -3 %
Eosino- 1.1%
Platelet- 156000
ESR- 120mm in 1st hour
MCV: 90.3
MCH: 33.2
MCHC:36.9
PBF: Nonspecific morphology
S. Electrolytes
Na-137 mmol/l
K-4.5 mmol/lCl: 106 mmol/lHCO3: 26 mmol/lCa- 9.3 mmol/lMg- 0.9 mmol/lPhosphate-3.7
Lipid profile:
TG: 176 mg/dl
T. Chol : 164 mg/dl
LDL: 95 mg/dl
HDL:36 mg/dl
LFT:
ALT: 34 iu/L
AST: 37 iu/L
S. Total protein: 86.2
S Alb: 26.9
RFT:
S. Creatinine: 0.8mmol/l
S Urea: 29 mmol/l
HbA1c: 7.2%
Sugar - Nil
Albumin – Nil
Ketone- Nil
Epi. cell: A few /HPF
Pus cell: 1-2 /HPF
RBC: Nil
URINE R/M/E
• Blood group: AB +ve• Iron profile:
– S Iron: 7.7– TIBC: 27.2– S ferritin: 312– T sat: 28 %
Chest X-Ray
NORMAL
ECG
Normal
X ray Lumbosacral spine
• Fracture L5 Vertebrae
MRI of Lumbosacral spine
• Collapse with altered signal intensity in L5 & focal altered signal intensity in L3, S1 vertebrae
• Central & paracentral disc bulge causing thecal sac indentation & bilteral lateral recess narrowing at L5/S1 level
MRI of Lumbosacral spine
MRI of Lumbosacral spine
MRI of Lumbosacral spine
MRI of Lumbosacral spine
• Appearance: clear• Protein: 66g/L• Sugar: 4.2mmol/L( Corresponding blood
glucose-6.8 mmol/l)• Cell count: • Total WBC : Nil• Total RBC: Nil• Bacterial antigen: Negative
• USG of whole abdomen: Normal• S. PSA: 1.13• S CEA: 2.74• CA-19.9: 26.4• Alpha feto protein: Normal
• Urine for bence jones protein: Absent
X ray Skull lateral view
• Multiple lytic lesion is present
Serum protein electrophoresis
• Monoclonal band(? M protein)
CT guided FNAC
• Tissue from lumbar vertebral body L5: Multiple myeloma
Bone marrow examination
• Grossly increased plasma cells almost completely replacing normal haemopoeitic cells replacing more than 80% of eisting marrow cells. The cells are distributed in sheets & clusters & include some immature forms consistent with multiple myeloma
Bone marrow examination
Final diagnosis:
• Diabetes Mellitus Type 2• Cauda equina syndrome due to multiple myeloma
Treatment:
Short acting insulin
Daily physiotherapy
Vitamin B
Calcium
I/V Dexamethasone
Blood Transfusion
Hospital course
Patient was immediately transferred to DMCH with prior consultation to haematologist & neurosurgeon for radiotherapy, antimyeloma therapy after confirmation of diagnosis.
Follow UP
Patient was advised to follow up in Neurology after 1 month for further clinical evaluation & management.
Discussion
Case Discussion
• Multiple myeloma is a condition of malignant plasma cell proliferation derived from a single B-cell lineage
• Making the diagnosis includes demonstrating M-proteins in either serum or urine, proving the presence of more than 10% of these malignant plasma cells in the bone marrow and observing the clinical manifestations of the disease in our patient
• Up to 30% of patients are diagnosed incidentally while being evaluated for unrelated problems, while another third are diagnosed following a fracture .
Case Discussion
• The incidence of bone pain from osteolytic lesions ranges from 58% to 66% of patients with myeloma
• Spinal cord compression following vertebral compression fractures or vertebral plasmacytomas comprises 5% of the presentations of multiple myeloma
• Recent articles revealed few case reports of plasmacytomas as initial presentations of multiple myeloma.
Cauda Equina Syndrome
Cauda equina syndrome refers to a characteristic pattern of neuromuscular and urogenital symptoms resulting from the simultaneous compression of multiple lumbosacral nerve roots below the level of the conus medullaris. These symptoms include low back pain, sciatica, saddle sensory disturbances, bladder and bowel dysfunction, and variable lower extremity motor and sensory loss.
Cauda Equina and Conus Medullaris Syndromes
Etiology
• Spinal trauma including fractures• Neoplasm, including metastases• Spinal infection/abscess• Idiopathic • Spinal hemorrhage• Multiple sclerosis• Spinal arteriovenous malformations• Late-stage ankylosing spondylitis• Neurosarcoidosis
History
Patients can present with symptoms of isolated cauda equina syndrome, isolated conus medullaris syndrome, or a combination. The symptoms and signs of cauda equina syndrome tend to be mostly lower motor neuron (LMN) in nature, while those of conus medullaris syndrome are a combination of LMN and upper motor neuron (UMN) effects
History
• Low back pain• Unilateral or bilateral sciatica• Saddle and perineal hypoesthesia or anesthesia• Bowel and bladder disturbances• Lower extremity motor weakness and sensory
deficits• Retention, Difficulty initiating micturition, Decreased
urethral sensation. urinary manifestations begin with urinary retention and are later followed by an overflow urinary incontinence.
Physical examination
• Muscle strength in the lower extremities is diminished.• Sensation is decreased to pinprick and light touch in a
dermatomal pattern corresponding to the affected nerve roots.
• Vibration sense may also be affected. • Muscle stretch reflexes may be absent or diminished in
the corresponding nerve roots. • Babinski reflex is diminished or absent.• Anal sphincter tone is patulous• Muscle tone in the lower extremities is decreased, which
is consistent with an LMN lesion.
Physical Examination
Diagnostic Considerations
• AIDP• Amyotrophic lateral sclerosis• Diabetic Neuropathy• Guillain-Barré Syndrome• Multiple sclerosis• Neoplasms of Spinal Cord• Neurosarcoidosis• Spinal Cord Infections• Traumatic
Complications
• Neurogenic bladder/bowel• Erectile dysfunction• Pressure ulcers• Osteoporosis• Chronic neuropathic pain• Spasticity/contractures• Recurrent urinary tract infections• Urethral stricture• Bladder calculi• Depression
• Corticosteroids• Skeletal Muscle Relaxants• AntimyelomaTherapy• Radiotherapy• Surgery• Vertebral Augmentation• Rehabilitation• Physical therapy• Occupational therapy• Orthotic/assistive devices may be needed
Radiotherapy
• External beam radiation therapy represents the treatment of choice for solitary plasmacytoma of the bone
• In MM, radiation to the spine is usually employed in patients with uncontrolled pain or in case of vertebral fracture or spinal cord compression
Surgical Decompression
• In acute compression of the conus medullaris or cauda equina, surgical decompression as soon as possible becomes mandatory
• In a more chronic presentation, decompression could be performed when medically feasible and should be delayed to optimize the patient's medical condition
Morbidity is determined by the underlying etiology.The prognosis improves if a definitive cause is identified and appropriate treatment occurs early in the course.