Acute renal failure in patients with axonal demyelinating polyneuropathy

Keywords

This is the case of a 50 year old male who presents with back pain, neck pain and left sensory-motor deficit


PERSONAL HISTORY

- No known allergies
- Toxic habits: smoking 2-3 cigars / day
- No hypertension, dyslipidemia or diabetes mellitus
- Hepatitis A 20 years ago. Blood donor.
- Hyperuricemia
- Surgical history: tonsillectomy.

Usual treatment:  allopurinol 300 mg per day

PRESENT ILLNESS

One week before admission, the patient has low back pain that did not improve with NSAIDs. Then gradually started with neck pain (left more than right).

On Friday 30/09/11 the patient insidiously began to feel a sensory deficit and weakness in left side of the body, so he decides to go to the ER. He had had general malaise for the last 3-4 days. No fever. No abdominal pain. No nausea or vomiting. No weight loss. No cough or expectoration. No other associated symptoms.

PHYSICAL EXAMINATION

- BP: 125/93 mmHg. HR: 67 bpm. RR: 16 bpm. T: 36 º C SaO2: 97%
- Appearance: Regular condition.
- Head and Neck: bilateral  large and painful lymphadenopathy.   No goiter.

- Left axillary adenopathy and bilateral inguinal lymphadenopathy. 


- Neurologic:

+Alert and oriented in time, space and person.

+No language impairments.

+No visual field deficit by confrontation.

+Bilateral facial palsy, greater degree on the left side.

+Other cranial nerves were normal.

+Left hemiparesis (4/5) and left facio-brachio-crural hemi-hypoesthesia.

+No motor or sensory deficits in right hemisphere.

+No limb differences in finger-nose maneuvers.

+EMR hypoactive (fourth) in upper limbs, abolished in lower limbs.

+PCR flexor bilateral.

+Signs of Kernig and Brudzinski doubtfully positive.

The remaining physical examination was normal


INVESTIGATIONS
- ANALYTICS:

CBC: mild leukocytosis (12.7 x10 ^ 9 / L) with left shift. Hb: 18.1 g / dL with Hct.: 53.7%. Normal Platelets.

Hemostasis: Fibrinogen in  639 mg / dL, the other parameters were normal

Biochemistry:  normal blood sugar. Urea: 52 mg / dL and creatinine: 1.74 mg / dL. Normal electrolytes. Normal transaminases. LDH: 1055 U / L. CRP: 38.6 mg / L. Total protein: 5.2 g / dL. Albumin: 2.20 g / dL.

Proteinogram: polyclonal immunoglobulin increase. Normal Immunoglobulin

Beta-2-microglobulin: 11050 mcg / L.

Normal thyroid function

- Urine:

Leucos + + +, ++++, protein + blood glucose + + +

Sed: hematies> 100 per field > 3% dysmorphic  >leu per field

urine protein 2215mg/dl

urine protein / urine creatinine ratio 125.852 mg / g

-Autoimmunity:

ANA, anti DNA, anti-ENA (anti-ENA antibodies studied include anti-SSA/Ro, anti-SSB/La, anti-RNP, anti-Sm, anti-and anti-Jo-1 Scl70.)

anti-smooth muscle,  anti-LKM,  anti-gastric parietal cel,

anti-mitochondrial,  antiphospholipid antibodies all  negative.

 

-ACE 15.10U / L normal

- Tumor markers:

Beta-2 microglobulin (7611mcg / l), AFP, CEA, CA 19.9, CA 15.3 and PSA and  BHCG all normal.

- Serology:

HIV, HCV and HBV negative

CMV IgM negativ and IgG positive,  CMV IgG 10.9 IU / ml (past infection)

Past infection by Epstein-Barr virus

Herpes simplex virus negative.

Total Treponema pallidum ab negative,

burgdorferi IgG + IgM detection negative

S.agalactiae Ag, H. influenzae type b Ag, S.pneumoniae Ag and  N. meningitidis Ag all negative

 

- Cerebrospinal fluid:
+03.10.11: Protein 269.0 mg / dl.  Albumin 163.00 mg/dl. Glucose .53.0 mg / dl.

CSF ADA 1U / L.

0 cells.

CMV IgG. Simple v.herpes IgG and IgG 1 +2 v.varicella-zoster. Absence of antibodies.


+20.10.11 Protein 235mg/dl  albumin 137mg/dl (2.90g/dl blood) glucose 52mg/dl, ADA 1U / L ,

cells 6 RBC

Ig G31mg/dl (normal in  blood) ACE normal and oligoclonal bands pending, CEF culture negative. 

- PPD (mantoux): negative (on seteroids)


-ECG: Sinus rhythm, 78 beats per minute: no acute changes in repolarisation.

RADIOLOGY:

-RX cervical spine: no significant alterations


-RX thorax: without significant alterations


- Cranial CT: no significant alterations


- Brain MRI 06.10.11: No significant changes are seen.


- Complete spinal MRI 20.10.11: No significant changes are seen


-Doppler ultrasound of the supraaortic arteries within normal limits. Numerous bilateral cervical lymphadenopathy striking for their numbers and some of them by their size,no specific by ultrasound (could be reactive to inflammatory / infectious).


-Renal ultrasonography, both kidneys have an increased echogenicity of the cortex. Moderate calyceal ectasia without pelvic dilatation is observed. In conclusion, findings consistent with medical renal disease.


- EMG 07.10.11:sensory-motor polyneuropathy mixed (axonal and demyelinating) with involvement at upper  and lower limbs which overlaps the existence of entrapment neuropathy of Median nerve,  bilateral carpal canal, moderate lesion extent. There is more sensitive involvement at the level of upper limbs (n. sural with milder involvement), and findings suggestive of demyelination (lengthening of waves f) findings in early stages of acute inflammatory demyelinating PNP (Guillain Barre Sd). We will check further in 7-10 days.


- ENG-EMG13.10.11: sensory-motor polyneuropathy mixed (axonal and demyelinating) with involvement of upper and lower limbs. Entrapment neuropathy of n. medium-level bilateral carpal canal, moderate lesion extent. Unchanged from previous study


-Thoraco-abdominal CT: Kidneys protruding, without signs of obstructive uropathy. Retroperitoneal and  multiple mesenteric lymphadenopathy (many of them sub-centimeter) should be evaluated in CT with iodinated contrast when kidney function improves.


- Chest and abdomen CT scan 14.10.11 Mild inflammatory changes and air bubbles in the left cervicolateral region, probably post-puncture. Slight right pleural effusion. Decrease of intra-and retroperitoneal lymphadenopathy persisting iliac lymph nodes, some of them still significant at the present time. Fray generalized abdominal fat and small amount of fluid in the pelvis, which can be related to fluid overload


- Renal biopsy (10/10/11). Provisional results:  4 glomeruli with endocapillary and mesangial hypercellularity  (IFD, and EM pendent). No crescents. Severe tubulointerstitial nephritis component probably secondary to NSAIDs. Congo Red Negative. (Pending final report )


-Transthoracic echocardiography 18.10.1:No dilated left atrium. Dilated LV (65 mm) with increased trabeculation and mild anterior hypokinesia lateroapical at that levels. No dilated right ventricle with preserved contraction. No MI. Pattern with delayed diastolic relaxation and filling pressures are not high. Trileaflet aortic valve without functional impairment. NO pericardial effusion. A level can be seen descending thoracic aorta nodule, but without concomitant flow alteration (artifact?)

PROGRESS:

During admission, the patient has a rapidly progressive anuric renal failure , requiring  acute intermittent hemodialysis and high dose corticosteroids. It was interpreted as an acute interstitial nephritis secondary to NSAIDs, superimposed on a glomerulonephritis. The renal function improved and he was dialysis free at discharge. We performed a fine needle aspiration of lymph nodes, showing only inflammatory cells. Lymph node biopsy was attempted, not getting material, as the lymph nodes were reduced in size with corticosteroid treatment.
In addition, he had asymptomatic self-limiting bradycardia (assessed during hemodialysis and telemetry) so that was raised in differential diagnosis between dysautonomia of Guillain Barré vs rhythm disorders secondary to cardiomyopathy.
As we found dilated cardiomyopathy we asked for cardiac MRI, currently pending. 

The patient at discharge is asymptomatic with almost complete resolution of sensory-motor syndrome and facial paralysis, with recovery of enough renal function to be dialysis free and normal heart rate, pending some results and complete study

MAIN DIAGNOSIS AT DISCHARGE REPORT


-Demyelinating polyneuropathy with axonal, peripheral facial diparesis  albumin-cytological dissociation of 3-week evolution
- Lymphadenopathy

- Acute tubulointerstitial nephritis secondary to NSAIDs.

-Possible prior chronic glomerulopathy (pending final report of renal biopsy)

-Dilated cardiomyopathy and episodes of bradycardia, pending completion of study.

 

 

DIFFERENTIAL DIAGNOSIS

We have considered:

 

-Lymphoproliferative process

-Systemic disease (amyloidosis, granulomatous disease, cryoglobulinemia ...)

-Infectious disease (TBC...)


Pending completion of the study.


TREATMENT AT DISCHARGE

Medication: 
- Omeprazole 20mg 
- Prednisone 80mg tablet each morning until 06.11.11 inclusive and then descending pattern.
- Almax 
- Cotrimoxazole 800/160mg tablet Monday-Wednesday-Friday
- Natecal D (calcium + vitamin D) one tablet at breakfast
- Amlodipine 5mg tablet at breakfast and another at dinner
- Neo Carduran 4mg 
- Chlorhexidine rinses after every meal
- Paracetamol 500mg every 8 hours if pain


- Absolute contraindication to use of NSAIDs (ibuprofen, diclofenac, dexketofropeno...)

TEST PENDING AT DISCHARGE

-Cardiac MRI 
-Muscle nerve biopsy
-Quantiferon
-cryoglobulins: 31-10-2011: 3,8 mg/dl (0,1% criocrit) type III cryoglobulinemia
-ACE in CSF (31-10-2011:4,3 U/L 1-6 normal)

 

This case is still open to discussion.

After obtaining the remaining complementary tests we will update the diagnosis

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