Related Experiment Video
Updated: May 23, 2025

Author Spotlight: Unveiling the Pathway Linking Obesity to Autoimmune Inflammation in Multiple Sclerosis
Published on: February 23, 2024
An interesting case of subacute sclerosing panencephalitis presenting with Balint's syndrome and dysautonomia
Swati Parida1, Nikhil Pandey2, Anand Kumar2
1Department of Neurology, Kalinga Institute of Medical Sciences, Bhubaneswar, Odisha, India.
Abstract:
Subacute sclerosing panencephalitis (SSPE) is a rare, progressive neurodegenerative disorder caused by persistent aberrant measles virus infection. It is characterized by behavioral changes, cognitive decline with deterioration in academic performance, visual dysfunction, focal or generalized seizures ,myoclonus, spasticity, mutism and akinesia ultimately leading to a vegetative state. Balint's syndrome, characterized by the triad of simultagnosia, optic ataxia, and oculomotor apraxia, as an initial presenting feature of SSPE is rare. Autonomic dysfunction in SSPE is attributed to central autonomic involvement, with decreased heart rate variability a predictor for arrhythmia and sudden cardiac death. We report an unusual case of a 22-year-old male presenting with features suggestive of Balint's syndrome. Myoclonus and cognitive decline appeared 6 months after the first onset of symptoms, along with autonomic dysfunction. Thereafter, rapid progression of symptoms was noted. Cerebrospinal fluid and electroencephalography after the first symptom onset were consistent with a diagnosis of SSPE. The patient ultimately succumbed to his illness. Thus we highlight atypical presentation of SSPE with autonomic dysfunction. A high index of suspicion is needed for prompt and timely intervention.
Insights
Subacute sclerosing panencephalitis (SSPE), a rare measles virus infection, typically presents with neurological decline. This case highlights an unusual SSPE presentation with Balint's syndrome and autonomic dysfunction, emphasizing the need for high diagnostic suspicion.
Area of Science:
- Neuroscience
- Virology
- Neuropathology
Background:
- Subacute sclerosing panencephalitis (SSPE) is a rare, fatal neurodegenerative disease resulting from persistent measles virus infection.
- SSPE typically manifests with progressive cognitive decline, behavioral changes, seizures, and motor deficits, ultimately leading to a vegetative state.
Observation:
- This report details an atypical SSPE case in a 22-year-old male initially presenting with Balint's syndrome.
- The patient subsequently developed myoclonus, cognitive decline, and autonomic dysfunction six months after symptom onset.
- Autonomic dysfunction in SSPE is linked to central autonomic pathway involvement and can predict cardiac complications.
Findings:
- Cerebrospinal fluid analysis and electroencephalography confirmed SSPE diagnosis.
- The patient experienced rapid symptom progression and ultimately succumbed to the illness.
- This case underscores the rare occurrence of Balint's syndrome as an initial SSPE manifestation.
Implications:
- Atypical presentations of SSPE, including Balint's syndrome and autonomic dysfunction, require a high index of clinical suspicion.
- Early diagnosis and intervention, though challenging, are crucial for managing SSPE.
- Understanding rare SSPE presentations improves diagnostic accuracy and patient outcomes.
More Related Videos
09:29Induction of Paralysis and Visual System Injury in Mice by T Cells Specific for Neuromyelitis Optica Autoantigen Aquaporin-4
Published on: August 21, 2017
07:50A Metadata Extraction Approach for Clinical Case Reports to Enable Advanced Understanding of Biomedical Concepts
Published on: September 20, 2018
Related Concept Videos
Disorders of the Autonomic Nervous System
Raynaud's disease, also known as Raynaud's...
Myasthenia Gravis: Overview and Treatment
These antibodies interfere with the function of the nicotinic receptors in three ways: by binding to the receptor and disrupting acetylcholine binding; by causing cross-linking of receptors which...
Acute Pancreatitis II: Clinical Manifestations and Management
The Parasympathetic Nervous System