ORIGINAL ARTICLE | https://doi.org/10.5005/jp-journals-10066-0093 |
Neuropathy in Thrombocytopenia: A Queer Complication
1,2Department of Physical Medicine and Rehabilitation, King George’s Medical University, Lucknow, Uttar Pradesh, India
Corresponding Author: Jyoti Pandey, Department of Physical Medicine and Rehabilitation, King George’s Medical University, Lucknow, Uttar Pradesh, India, Phone: +91 7355606542, e-mail: drjyotipandey7@gmail.com
How to cite this article Pandey J, Gupta AK. Neuropathy in Thrombocytopenia: A Queer Complication. Indian J Phys Med Rehab 2020;31(3):63–65.
Source of support: Nil
Conflict of interest: None
ABSTRACT
Mild to serious bleeding can occur as the major signs and symptoms of thrombocytopenia. Bleeding can be internal or external. Signs and symptoms can appear suddenly or over time. Mild thrombocytopenia often has no signs or symptoms. Severe thrombocytopenia can cause bleeding in almost any part of the body. External bleeding usually is the first sign of a low platelet count. Extrinsic compression due to hematoma, intraneural bleed, and immune-mediated nerve injury has been reported in the literature as mechanisms of neuropathy. Neuropathy due to thrombocytopenia is a rare complication with challenging manifestations. We report a case series of patients with dengue hemorrhagic fever (DHF) and idiopathic thrombocytopenic purpura (ITP) who had difficulty in standing and walking. One of the patients regained his muscle strength following the treatment and his quality of life was improved significantly.
Keywords: Dengue hemorrhagic fever, Electrical stimulation, Idiopathic thrombocytopenic purpura, Peripheral neuropathy, Thrombocytopenia.
INTRODUCTION
The development of peripheral neuropathy in hemorrhagic disorders is predominantly due to the formation of hematoma in adjacent structures leading to extraneural compression. Intraneural hemorrhage and immune-mediated neuropathy are rarely reported to cause neuropathy. In this case series, we present a patient diagnosed with dengue hemorrhagic fever (DHF) and another having idiopathic thrombocytopenic purpura (ITP), both of them developed peripheral neuropathy. Severe dengue and DHF coexist with thrombocytopenia, vascular leakage, and hypotension. There are few unusual manifestations of dengue fever that are often missed and sometimes difficult to apprehend the case collectively. Concurrent ITP associated with neuropathy is an extremely rare condition. Neuropathy varies from mono- to polyneuropathy, symmetric or multiplex, and in time of development, acute or chronic. If the intraneural hemorrhage occurs, the presentation may be acute and asymmetric.
CASE DESCRIPTIONS
Case 1
A 48-year-old, male government employee presented in our OPD with progressive numbness and weakness in his right lower limb for 1 week following dengue fever, making him unable to stand and walk without support. The patient was taking treatment (platelet transfusion and analgesics) for dengue fever with thrombocytopenia (Hb: 7 g/dL, WBC: 7,000/μL, platelets: 7,000/μL, INR-1.0). There was no history of any associated comorbidities. On examination, there was a weakness of right hip flexors, extensors (grade 4/5), hip abductors and adductors (grade 5/5), and knee extensor (grade 2/5), knee flexors, dorsiflexors, and plantarflexors 5/5 on MRC (medical research council). Right hip flexion, extension, abduction, and external rotation movements were painful. There was a loss of touch, temperature, and pain sensations in the distribution of dermatome L2, L3, and L4 on the right lower limb. Right knee reflexes could not be elicited due to pain while normal on the left side, bilateral planters were downgoing. USG lower abdomen showed a mixed echoic collection of size 12.9 × 7.9 cm in right iliopsoas muscle. MRI of the lumbosacral spine with a screening of pelvis with both hips revealed a well-defined hypointense lesion (measuring 13.5 cm SI × 7.5 cm TR × 7 cm AP) involving right iliacus muscle along with interspersed well defined small area of hyperintensity, the lesion was compressing and displacing psoas muscles anteromedially (Fig. 1). CECT abdomen also showed iliopsoas hematoma with mild fat stranding (Fig. 2). On NCS, the amplitude of SNAP and CMAP of the right femoral and lateral cutaneous nerve of the thigh was significantly decreased. Surface EMG showed denervation of the right quadriceps femoris. Laparoscopic evacuation of 50 mL clots under general anesthesia with the placement of tube drain and vacuum drain was performed at some hospitals. Electrical stimulation to the quadriceps muscle at its motor points and strengthening exercises were started after the procedure in our department. The patient was also prescribed knee orthosis to assist standing and walking. Quadriceps showed gradual recovery with the strength to grade 4/5 and full functional recovery at 10-month follow-up. The patient has also been prescribed pregabalin and vitamin B12 supplements for neuropathic pain which gradually subsided in an 11-month follow-up visit.
Fig. 1: MRI LS spine and pelvis shows well-defined hypointense lesion (measuring 13.5 × 7.5 × 7 cm) involving right iliacus muscle along with an interspersed well-defined small area of hyperintensity, lesion compressing psoas muscle anteromedially
Fig. 2: CECT abdomen shows iliopsoas hematoma with mild fat stranding
Case 2
Another 40-year-old man visited our OPD with difficulty in walking for 5 months. The weakness of the leg was progressive. Also, he had complained of paresthesia and a burning sensation in his right leg. The patient was a diagnosed case of ITP, 1 year 9 months ago. At the diagnosis, he had splenomegaly, purpura, and low platelet count, reaching 9,000 pL/mm3, Hb: 12.2 mg/dL; leukocytes: 7,400 cells/mm3, and normal coagulation. The urine was normal. He was treated with steroids and IVIG for 6 months. He had no past medical history other than this. On examination, he had weakness in right lower limbs with power in hip flexors (4/5), right knee extensors (4/5), dorsiflexor right (3/5), and left (4/5) on manual muscle testing. The ankle reflexes were diminished. He presented with hypoesthesia in L4, 5. The rest of the neurological examination was normal. Routine blood test results showed no abnormalities. Rheumatoid factor, antinuclear antibodies, C-reactive protein, anti-double-stranded DNA, anti-phospholipid antibodies, antibodies against HIV-1 and HIV-2, hepatitis B virus, and hepatitis C-virus were not remarkable. Serum levels of vitamin B12 were within the normal limits. Lumbar puncture showed an elevated value of protein in CSF. There was no evidence of extrinsic nerve compression on imaging of the extremities. NCS showed peripheral neuropathy, more motor than sensory with a predominant axonal component. The alteration was more severe and asymmetric at the distal part of the legs. There was an evidence of denervation at the distal leg muscles on EMG. He was treated with high-dose steroids intravenously and was sent to our side. Here, strengthening exercises and electrical stimulation of quadriceps and dorsiflexors were given. The patient showed a slight improvement in the symptoms.
DISCUSSION
The clinical manifestations of dengue infection can be variable in presentation. Intramuscular hematomas are rare complication. Ganeshwaran et al.,1 Ammer et al.,2 and Ganu and Mehta3 have reported cases of DHF with intramuscular hematomas in the rectus muscle, psoas, and iliopsoas, respectively. In our patient, there was a hematoma in the iliopsoas muscle.
Although the pathogenesis of bleeding is poorly understood, it is mostly due to the immune response of the host to dengue virus infection which leads to disseminated intravascular coagulation, endothelial dysfunction, increased vascular permeability, thrombocytopenia, and platelet dysfunction. Considering the anatomical fact, the fascia overlying iliacus muscle and the femoral nerve are strong and almost non-stretchable. There will be ischemic damage to the femoral nerve due to hematoma in the iliopsoas gutter as the nerve has poor blood supply in this region. Also, the hematoma may reach the femoral canal and compress the femoral nerve against the inguinal ligament. Some literature has also demonstrated that the iliacus muscle hematoma is mainly responsible for femoral nerve palsy and not the hematoma in the psoas muscle.4 As iliacus muscle hematoma never drains spontaneously and induces chronic compression on the femoral nerve, surgical intervention (evacuation) becomes essential for nerve recovery. Decompression of the femoral nerve thus allows functional recovery. In our patient, laparoscopic evacuation of the blood clot for decompression of the femoral nerve was done. Following the procedure, the patient had a speedy recovery.
Neuropathy with coexisting ITP is an extremely rare condition. Neuropathy varies from mono- to polyneuropathy, symmetric or multiplex, and in time of development, acute or chronic. If the presentation is acute and asymmetric, it happens mainly due to intraneural hemorrhage.5 Immune-mediated neuropathy and vasculitis leading to neuropathy have also been reported in patients with ITP. In our patient, nerve conduction studies were suggestive of axonal neuropathy of the right common peroneal nerve. There was no evidence of extrinsic nerve compression on imaging of the extremities. Although there was no evidence of intraneural bleeding on imaging, it is possible that small intraneural lesions can be missed. We guess that intraneural hemorrhage was the cause of neuropathy in our patient and it was temporally related to the active hemorrhagic phase of ITP. There was no evidence to suggest an autoimmune disorder. The patient was on steroids and IVIG therapy. Although the recovery was slower compared with the first case, the patient was receiving electrical stimulation and performing strengthening exercises of the weak muscles. Several studies have documented the therapeutic benefit of electrical stimulation on muscle-fiber regeneration in LMN denervation.6–8
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