Dataset Information

Uraemic brainstem encephalopathy mimicking ocular myasthenia: a case report.

ABSTRACT:

Background

Uraemia causes a generalised encephalopathy as its most common neurological complication. Isolated brainstem uraemic encephalopathy is rare. We report a case of fatigable ptosis and complex ophthalmoplegia in brainstem uraemic encephalopathy.

Case presentation

A 22-year-old Sri Lankan man with end stage renal failure presented with acute onset diplopia and drooping of eyelids progressively worsening over one week. The patient had not complied with the prescribed renal replacement therapy which was planned to be initiated 5 months previously. On examination, his Glasgow coma scale score was 15/15, He had a fatigable asymmetrical bilateral ptosis. The ice-pack test was negative. There was a complex ophthalmoplegia with bilateral abduction failure and elevation failure of the right eye. The diplopia did not worsen with prolonged stare. The rest of the neurological examination was normal. Serum creatinine on admission was 21.81 mg/dl. The repetitive nerve stimulation did not show a decremental pattern. Magnetic resonance imaging (MRI) of the brain demonstrated diffuse midbrain and pontine oedema with T2 weighted/FLAIR hyperintensities. The patient was haemodialyzed on alternate days and his neurological deficits completely resolved by the end of the second week of dialysis. The follow up brain MRI done two weeks later demonstrated marked improvement of the brainstem oedema with residual T2 weighted/FLAIR hyperintensities in the midbrain.

Conclusions

Uraemia may rarely cause an isolated brainstem encephalopathy mimicking ocular myasthenia, which resolves with correction of the uraemia.

SUBMITTER: Ruwanpathirana P

PROVIDER: S-EPMC11010437 | biostudies-literature | 2024 Apr

REPOSITORIES: biostudies-literature

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Publications

Uraemic brainstem encephalopathy mimicking ocular myasthenia: a case report.

Ruwanpathirana Pramith P Chang Thashi T

BMC neurology 20240412 1

<h4>Background</h4>Uraemia causes a generalised encephalopathy as its most common neurological complication. Isolated brainstem uraemic encephalopathy is rare. We report a case of fatigable ptosis and complex ophthalmoplegia in brainstem uraemic encephalopathy.<h4>Case presentation</h4>A 22-year-old Sri Lankan man with end stage renal failure presented with acute onset diplopia and drooping of eyelids progressively worsening over one week. The patient had not complied with the prescribed renal r ...[more]

PMID: 38609854

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Project description:BACKGROUND:The imaging findings of hypoglycemic encephalopathy can be considerably similar to those of ischemic infarction or toxic leukoencephalopathy. We demonstrated unusual magnetic resonance (MR) imaging features of hypoglycemic encephalopathy which can be confused with other pathology both on imaging and acute clinical presentation. The diffusion-weighted imaging (DWI) and apparent diffusion coefficients (ADC) map findings in our case further supports the hypothesis of hypoglycemia-induced "excitotoxic injury" of glial cells and myelin sheath that might protect neuron axons from intracellular edema and irreversible damage. CASE PRESENTATION:A 72-year-old woman presented with poor appetite and was initially drowsy at home; the symptoms progressed to loss of consciousness accompanied by mild incontinence. The initial glucose level was 44 mg/dL, but no nausea, vomiting, fever, or cold sweating was reported. Physical examination after intravenous glucose supplementation revealed the absence of focal neurological signs, facial palsy, and tongue or eye deviations. The images obtained 24 h after symptoms onset revealed symmetrical hyperintensities on DWI (b-value: 1000) associated with hypointensities on ADC map along the corticospinal tract, from the levels of the cerebral peduncle and the posterior limbs of the internal capsule to the level of the corona radiata, which may mimic the imaging findings of acute ischemic infarction or amyotrophic lateral sclerosis. The patient received sliding-scale insulin therapy and rehabilitation, and she recovered consciousness without motor function deficits after 1 month. Moreover, repeat DWI and ADC map showed the complete disappearance of the lesions. CONCLUSIONS:In the phenomenon of excitotoxic injury, axons could be protected from intracellular edema and irreversible damage, which may explain the reversible clinical symptoms and imaging abnormality after controlling for blood glucose because of the preserved motor axon. The diagnosis of acute symptomatic hypoglycemic encephalopathy through clinical and imaging features can be challenging. It is crucial to differentiate it from ischemic encephalopathy since the management and clinical outcome are different.

Project description:BackgroundCoexistence of thyroid-associated ophthalmopathy (TAO) and ocular myasthenia gravis (OMG) is very rare. Little is known about the orbital histopathology associated with this condition. The authors reported a case of TAO coexisting with OMG and explored the histopathologic changes in extraocular muscles.Case presentationA 32-year-old man complaint of bilateral proptosis for 2?years. The patient was documented with a history of OMG and was treated with blepharoplasty to correct ptosis 3?years prior to presentation. Physical examination revealed right upper eyelid retraction resulting from the eyelid surgery. Computed tomographic scan demonstrated bilateral enlargement of the extraocular muscles. Thyroid function test confirmed hyperthyroid status. The patient was diagnosed with TAO (clinical activity score?=?2/7) coexisting with OMG. Orbital decompression surgery reduced proptosis but resulted in new onset of left upper eyelid retraction because of the increased motor impulses to sustain eyelid elevation. Extraocular muscles were sampled during surgery and subjected to histopathologic stain. The stain results were analyzed against samples from age-, gender- matched TAO and control (non-TAO non-OMG) subjects. The measurement of myofiber size and glycosaminoglycan/collagen-occupied area was repeated in 3 randomly chosen fields of each slide. The variation of myofiber size was larger in the TAO?+?OMG (289.9?±?142.5??m2) samples than the TAO (544.1?±?160.6??m2) and control (157.0?±?47.7??m2) samples. Glycosaminoglycan was more abundant in the TAO?+?OMG (48.8?±?12.2%) samples than the TAO (28.4?±?3.6%) and control (3.3?±?0.8%) samples. Collagen fibers accumulated in the TAO (60.5?±?6.4%) samples but not in the TAO?+?OMG (36.1?±?4.3%) and control (33.9?±?2.7%) samples. Typical OMG changes were observed in the TAO?+?OMG samples but not in the TAO and control samples. These changes included central nuclei, aggregation of mitochondria and fiber type grouping. The histopathologic findings of TAO?+?OMG were summarized as inhomogeneously enlarged muscle fibers and predominantly endomysial accumulation of glycosaminoglycan.ConclusionThis study highlights the possibility of TAO coexisting with OMG and demonstrates the histopathologic features in this rare condition.

Dataset Information

Uraemic brainstem encephalopathy mimicking ocular myasthenia: a case report.

Background

Case presentation

Conclusions

Publications

Uraemic brainstem encephalopathy mimicking ocular myasthenia: a case report.

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