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Eye-of-the-Tiger Sign with an Unexpected Pathological Diagnosis
Elena Natera-Villalba1, Juan Carlos Martínez-Castrillo1,2, José Luis López-Sendón Moreno1,2
1Neurology Department Hospital Universitario Ramón y Cajal Madrid Spain.
Background:
Clinical diagnosis of atypical parkinsonisms may be challenging. The eye-of-the-tiger sign on brain MRI, typical of neurodegeneration with brain iron accumulation, has been anecdotally observed in cases clinically diagnosed as atypical parkinsonisms.
Objectives:
To show how clinical syndromes and even neuroimaging sometimes may lead the neurologist to a misunderstanding, just as to emphasize the important role of pathology to establish the final diagnosis in these cases.
Methods:
Clinico-pathological case.
Results:
A 67-year-old-woman presented with progressive painful stiffness and allodynia in her left arm. On examination, she presented parkinsonism without tremor with greater involvement of left limbs. She developed dystonia, with myoclonic tremor and hypoesthesia involving her left arm, as well as an impairment of balance with falls, a significant axial involvement with disabling rigidity, supranuclear gaze abnormalities, facial dystonia, dysphonia, severe dysphagia, and anarthria. There was no response to levodopa. Syndromic diagnosis and findings on neuroimaging are discussed. Afterwards, the underlying pathology is revealed.
Conclusions:
We present the first case of neuropathologically confirmed multiple system atrophy with the eye-of-the-tiger sign on brain MRI. The presence of supranuclear vertical gaze palsy further complicated a correct clinical diagnosis. A pathological postmortem study remains essential to establish a definite diagnosis in atypical parkinsonisms.
Insights
The eye-of-the-tiger sign on MRI can mimic atypical parkinsonisms. Neuropathological confirmation is crucial for accurate diagnosis in complex neurological cases.
Area of Science:
- Neurology
- Neuroimaging
- Pathology
Background:
- Atypical parkinsonisms present diagnostic challenges.
- The eye-of-the-tiger sign on MRI is associated with neurodegeneration with brain iron accumulation.
- This sign has been anecdotally noted in atypical parkinsonism diagnoses.
Purpose of the Study:
- To illustrate diagnostic challenges in neurology.
- To highlight how clinical presentation and neuroimaging can mislead.
- To emphasize the definitive role of pathology in diagnosing complex cases.
Main Methods:
- Presents a clinico-pathological case study.
- Details clinical presentation, neurological examination, and MRI findings.
- Correlates clinical and imaging data with postmortem pathological findings.
Main Results:
- A 67-year-old woman showed parkinsonism, dystonia, and autonomic dysfunction.
- MRI revealed the eye-of-the-tiger sign.
- Clinical diagnosis was complicated by supranuclear gaze abnormalities; pathology revealed multiple system atrophy.
Conclusions:
- This is the first reported case of neuropathologically confirmed multiple system atrophy with the eye-of-the-tiger sign.
- Supranuclear vertical gaze palsy complicated the clinical diagnosis.
- Postmortem pathological study is essential for definitive diagnosis of atypical parkinsonisms.
