CME INDIA Case Presentation by Dr. Suprotim Ghosh, Dr. Subhadeep Basu, Dr. Rajesh Kumar Singh – Under Guidance of Professor Shibendu Ghosh, Professor of Medicine, JIMSH, Kolkata, Vice-president API headquarters.
CME INDIA Case Study
How Presented?
- A 35-year-old obese woman presented with fever, diarrhea and headache with sudden visual loss and slurred speech.
- She developed weakness in her right upper and lower limbs.
- She was a known diabetic on irregular medication.
What was found on examination?
- Patient was febrile, conscious, oriented, dysarthric, absent meningeal signs.
- Neurologically:
- Cranial nerves: Left II, III, IV, VI, VII palsy.
- Sensory loss along left maxillary (V2).
- Right hemiparesis, with plantar extensor.
Investigations
- Blood: Polymorph leukocytosis, hyperglycemic, hyperlipidemia.
- MRI (Fig. 2): Infarcts in left parietal, temporal, frontal region;
- MRA (Fig. 3): occlusion in left ICA with poor visualized left MCA.
- Echocardiogram normal.
- DSA was done.
Later On
- She developed painful deglutition with sores in mouth.
- O/E: Ulcers in oral, nasal cavity.
- CT- PNS (Fig. 4 A&B): Pansinusitis, mucosal thickening.
- Initial microscopy (Fig. 4F) hyphae with branching.
- Tissue biopsy (Fig.4E) later confirmed Mucormycosis.
Final Diagnosis
- Multiple cranial nerve palsy with embolic infarcts in a diabetic patient with rhino-cerebral Mucormycosis.
Management
- Provisionally diagnosed with stroke and sepsis, treatment started with antibiotics, statin; antiplatelets added as MRI showed non-hemorrhagic infarcts.
- Mucormycosis was treated with Liposomal Amphotericin B, renal function monitored. Anticoagulant LMWH was added and antibiotics upgraded to contain cavernous sinus infection and thrombosis.
Follow Up
- Patient turned clinically better.
- She was referred to maxillo-facial surgery setup on D6 for surgical debridement of the affected part.
CME INDIA Learning Points
- Mucormycosis is a term used to refer to any fungal infections of the order Mucorales. It belongs to class Zygomycetes. Rhinocerebreal Mucormycosis is a rare but fatal infection of nasal cavity and sinuses.
- Rhino-orbito-cerebral Mucormycosis is known to exist in two forms, the well-known acute form and the less well-recognized chronic form.
- Acute rhinocerebral Mucormycosis is an opportunistic but fulminant fungal infection of nose, sinuses, orbit and cranial structures. It aggressively spread to orbits and cranium within days.
- It is well known that in advanced disease chemosis, ptosis, proptosis, ophthalmoplegia and blindness and multiple cranial nerve palsy (function of the cranial nerves II, III, IV, V and VI may be lost or impaired.
- Depending on the immunological status of the host, the disease may manifest in six different ways depending on the affected site:
- Rhinocerebral.
- Pulmonary.
- Cutaneous.
- Gastrointestinal.
- Central nervous system.
- Miscellaneous.
- Patients with diabetes mellitus usually have the rhinocerebral and pulmonary forms of disease.
- Patients who are malnourished usually have the gastrointestinal forms of disease.
- Poor response to prolong antibiotic treatment in diabetic should raise the suspicion of fungal disease.
- Blood-stained nasal discharge is usually a warning sign.
- Cranial nerve palsy with eye involvement in diabetics may be an alerting sign. Thorough history, supported by detailed examination confirm by biopsy supplemented by CT along with surgical and medical management are required.
- Bell’s palsy is known to occur in diabetics however multiple cranial nerve palsy should raise suspicion of Mucormycosis.
- Opportunistic infection with fungi in nasal sinuses is rare, yet it is being reported nowadays in the COVID-19 pandemic. COVID-19 infection has consequences in patients who are either immunocompromised, with uncontrolled diabetes, and who are treated for long periods with high doses of steroids.
- COVID-19 infection in recent past and diabetes (pre-existing or new onset; diagnosed during COVID-19) are the two most important risk factors)
- ROCM though predominantly unilateral, often becomes a bilateral disease with delay in diagnosis.
- Neuroaxis involvement has been characterized by myriad of clinical symptoms and signs among which proptosis/ptosis, external ophthalmoplegia, diminution of vision/loss of vision, stroke, facial numbness and facial palsies were most common.
- Mucormycosis is a deadly disease carrying more than 50% mortality despite standard care. The morbidity in survivors is even more. The treatment is as deadly as the disease. The multidisciplinary approach, expensive medicines with high adverse effects, a prolonged course of illness etc. makes it most challenging to manage.
- Rapidity of dissemination of mucormycosis is an extraordinary phenomenon and even a delay of 12 h in the diagnosis could be fatal, the reason 50%.
- DM remains the leading risk factor associated with mucormycosis globally.
- While long term use of corticosteroids have often been associated with several opportunistic fungal infection including aspergillosis and mucormycosis, even a short course of corticosteroids has recently been reported to link with mucormycosis especially in people with DM.
- A cumulative prednisone dose of greater than 600 mg or a total methyl prednisone dose of 2–7 g given during the month before, predisposes immunocompromised people to mucormycosis.
CME INDIA Tail Piece
References
- Dyer O. Covid-19: India sees record deaths as “black fungus” spreads fear. BMJ. 2021;373:n1238. doi: 10.1136/bmj.n1238
- Sano T, Kobayashi Z, Takaoka K, Ota K, Onishi I, Iizuka M, Tomimitsu H, Shintani S. Retrobulbar optic neuropathy associated with sphenoid sinus mucormycosis. Neurol Clin Neurosci. 2018;6(5):146–147. doi: 10.1111/ncn3.12216.
- Dubey, Souvik et al. “COVID-19 associated rhino-orbital-cerebral mucormycosis: An observational study from Eastern India, with special emphasis on neurological spectrum.” Diabetes & metabolic syndrome vol. 15,5 (2021): 102267. doi:10.1016/j.dsx.2021.102267
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