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We describe a 36-year-old man with subacute sclerosing panencephalitis (SSPE) presenting with chorioretinitis two years before onset of other neurological features. He had neither myoclonus nor the typical EEG features of SSPE. The diagnosis was confirmed in the appropriate clinical setting by detecting elevated measles antibody titres in cerebrospinal fluid and serum. Clinicians should consider SSPE among the differential diagnoses in chorioretinitis. This is particularly so if there is macular or perimacular involvement with concurrent involvement of the optic nerve in young patients, even without other characteristic neurological symptoms.

Background: Progressive neurological genetic diseases are not rare. They cause psychosocial damages to its victims. This article focuses on common psychosocial issues faced by those from the developing world. Methods: A multicentre observational survey of 246 patients from teaching hospitals in Sri Lanka. Participants were clinically and genetically confirmed by neurologists and the Interdisciplinary Centre for Innovation in Biotechnology and Neuroscience (ICIBN) respectively from 2014 to 2018. Convenience sample with random geographical distribution. Factors were equally weighted. ANOVA, Student’s t-test and chi-square analysis were used. Statistical Software R Statistics—version 3.5 and one-sample t-test with CI = 95% was used. This study meets the ethical guidelines of the local institutional review boards which are in compliance with the Helsinki Declaration. Results: Sample included 184 males and 62 females of 3–76 years with either Duchenne muscular dystrophy (n=121), spinocerebellar ataxia ( n = 87) or Huntington disease ( n = 38). Mean income of the affected is lower than the standard average monthly income ( P ≤ .001). Consultation visits depend on the monthly income (CI 20421.074–34709.361; P ≤ .001). Conclusion: Poverty is inversely proportionate to the patients’ living conditions. As developing countries are financially challenged, it is a societal challenge to rebuild our values to enhance their living status.

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Background: A presentation of upper motor and lower motor clinical signs without sensory impairment may suggest Amyotrophic lateral sclerosis, especially when it involves limbs and bulbar regions. However, co-existence of two or more unrelated pathologies can give rise to a clinical picture similar to Amyotrophic lateral sclerosis. Case presentation: A 45 year old lady presented with slowly progressive right upper limb weakness for 6 months and mild left upper limb weakness for 2 months associated with muscle twitching. Her family members have noticed a change in character of her speech for few weeks. She couldn’t recall a radicular pain at any given time. On examination, her right deltoid was wasted with numerous fasciculations over right proximal muscles. Right shoulder movements were weak. Her left deltoid was slightly wasted and fasciculations were appreciated. Her both biceps and supinator jerks were normal with exaggerated triceps jerks and positive Hoffman sign. Sensory and proprioception examinations were unremarkable. Both lower limbs were hypertonic with exaggerated knee and ankle jerks. Ankle clonus was absent and plantar responses were equivocal. Her tongue was deviated to right side with fascicuations and wasting of right side. Palatal movements and Jaw jerk were normal. She was clinically diagnosed as possible amyotrophic lateral sclerosis. Electromyography showed denervation changes in deltoids and biceps with right predominance and right genioglossus muscle too showed denervation changes. Magnetic resonant imaging of brain stem was undertaken especially because her tongue wasting and fasciculations were unilateral and it showed a right sided hypoglossal schwannoma. Magnetic resonant imaging of cervical spine revealed degenerative disk disease with mild cord compression and exit foramina narrowing at multiple levels. Conclusions: Pure motor symptoms with a mixture of upper motor and lower motor signs may suggest a clinical diagnosis of Amyotrophic lateral sclerosis. However, when there are rare manifestations of a relatively common disease, such as unilateral tongue involvement as in the above scenario, a higher degree of clinical suspicion is needed to think of a dual pathology. Keywords: Hypoglossal schwannoma, cervical myeloradiculopathy, Amyotrophic lateral sclerosis, dual pathology.

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