|Year : 2015 | Volume
| Issue : 2 | Page : 109-111
The "hummingbird" lady: A case report on progressive supranuclear palsy
Mosam V Phirke, Era S Dutta, Avinash Desousa, Nilesh B Shah
Department of Psychiatry, Lokmanya Tilak Municipal Medical College and General Hospital, Mumbai, Maharashtra, India
|Date of Web Publication||18-Jan-2016|
Era S Dutta
Lokmanya Tilak Municipal Medical College and General Hospital, Sion, Mumbai - 400 022, Maharashtra
Source of Support: None, Conflict of Interest: None
Progressive supranuclear palsy (PSP) is a rare neurodegenerative disease, which tends to be progressive, causing weakness of the structures in the brain stem that controls eye movements. PSP falls under the rubric of Parkinsonism plus syndromes that are a group of heterogeneous degenerative neurological disorders that differ from the classical idiopathic Parkinson's disease. PSP is often underreported, making it important for clinicians to be aware of this disorder. Here we report a case of PSP, which presented primarily with speech disturbances and recurrent falls due to postural instability. On investigating, the magnetic resonance imaging (MRI) revealed a classical sign diagnostic of PSP.
Keywords: Humming bird, penguin sign, progressive supranuclear palsy (PSP)
|How to cite this article:|
Phirke MV, Dutta ES, Desousa A, Shah NB. The "hummingbird" lady: A case report on progressive supranuclear palsy. J Geriatr Ment Health 2015;2:109-11
|How to cite this URL:|
Phirke MV, Dutta ES, Desousa A, Shah NB. The "hummingbird" lady: A case report on progressive supranuclear palsy. J Geriatr Ment Health [serial online] 2015 [cited 2019 Nov 19];2:109-11. Available from: http://www.jgmh.org/text.asp?2015/2/2/109/174280
| Introduction|| |
Progressive supranuclear palsy (PSP) is a rare neurodegenerative disease affecting the brain stem, basal ganglia, and cerebellum.  It is also named Steele-Richardson-Olszewski disease.  Although the exact cause of this disease is not known, evidence suggests it is due to abnormal deposition of tau protein in neuronal tissues, thereby making it a tauopathy. An overall prevalence of 1.39 cases per 100,000 populations was seen in a population-based study carried out in New Jersey, along with a slight male preponderance. The adjusted prevalence ratio among patients older than 55 years is 7 in 100,000, making it a rare disease.
PSP is a disorder of middle or late age, affecting both men and women almost equally after the sixth decade of life, with a prevalence rate of 5-6 per 100,000 and median survival of 7-12 years after diagnosis.  The presentation may vary from complaints of multiple falls directed backwards, dysarthria, downward gaze palsy, axial rigidity, and eventually cognitive impairment. , Magnetic resonance imaging (MRI) is helpful in making the diagnosis.
Here, we report a case of early onset PSP presenting with repetition of words, abnormal eye movement, and repeated falls.
| Case report|| |
A 60-year-old Muslim widow from rural North India presented to the outpatient department with repetition of words spoken to her and fixed upward gaze with onset 2 years history prior to presentation. The patient suffered multiple falls at home due to inability to look downwards. The patient was unable to take care of herself compounded with slowness in all activities. She became aloof and did not interact with anyone. She had trouble swallowing during feeding and needed assistance. Sleep and appetite were impaired with no history of tremulousness, hallucinations, forgetfulness, and urinary incontinence. Family history was insignificant. No treatment had ever been sought.
On general examination vital parameters were normal. Multiple marks on the head due to recurrent falls were noticed while systemic examination was normal.
On examining the nervous system, pupils were bilaterally equal and reactive to light; eyes remained fixed in a constant staring upward gaze, [Figure 1] depicting supranuclear horizontal and vertical gaze palsies. Dolls eye reflex was present. Axial rigidity in form of nuchal rigidity, stiffness of the back muscles, lead pipe rigidity in all four limbs, and grade 4 power existed. All deep tendon reflexes were brisk. Bradykinesia with difficulty in walking was present. Glabellar tap, palmomental reflex, and suckling reflex (primitive reflexes) were positive.
On mental status examination, the patient was semiconscious, with impaired attention, and she kept repeating the words said to her. No symptoms suggestive of Tourette's syndrome were present. It was difficult to conduct a formal examination.
Routine investigations were within normal limits. MRI brain showed a characteristic "Humming Bird Sign" in axial view along with atrophy in the frontal lobe [Figure 2].
|Figure 2: Inset — humming bird outset — MRI showing the comparative humming bird|
Click here to view
Treatment provided was mainly symptomatic in the form of citicoline (400 mg twice daily), piracetam (800 mg twice daily), baclofen (30 mg twice daily) for rigidity, and clonazepam (0.5 mg at night) for sleep. Levodopa + carbidopa (110 + 10 mg in two divided doses) was added by the neurophysician. Physiotherapy was started. There was minimal improvement after 2 weeks of treatment. Sleep improved slightly but communication remained poor. The prognosis was explained to the relatives.
| Discussion|| |
The clinical research criteria given by the National Institute of Neurological Disorders and Stroke (NINDS-SPS) for the diagnosis of PSP was used to make a diagnosis in this case. , The Humming Bird Sign or Penguin Sign is radiologically diagnostic of PSP. Humming bird is a small bird with a characteristically slender long beak or bill [Figure 3]. The bird's body is represented by atrophy of midbrain tegmentum seen in mid-sagittal T1-weighted images. It shows a 100% sensitivity in diagnosing PSP.  The usual upwardly convex outline of the superior aspect of the midbrain is flattened or concave.  A diagnosis of PSP was made after eliminating other Parkinson's diseases. PSP often overlaps with Parkinson's disease, but there are differences such as a poor response to levodopa, distinctive pathological characteristics, and poor prognosis. ,,
To our dismay, no effective treatment guidelines are available for PSP. A few recent studies have pointed toward the use of rivastigmine for cognitive enhancement and zolpidem to improve sleep, but these studies are anecdotal in nature. ,, The role of a multispecialty treatment team is a must in the management of this complex disorder. PSP often goes underreported or misdiagnosed and better understanding of PSP can help the clinicians identify the condition. The role of neuroimaging as a tool in diagnosis is crucial along with one's clinical judgment.
|Figure 3: Humming bird sign: Mid-sagittal T1-weighted sequence MRI brain showing atrophy of the midbrain tegmentum, with a relatively preserved pons, decreased midbrain-to-pons ratio with a superior aspect concavity, resembling the head and body, respectively, of a humming bird|
Click here to view
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Morris HR, Wood NW, Lees AJ. Progressive supranuclear palsy (Steele-Richardson-Olszewski disease). Postgrad Med J 1999;75:579-84.
Steele JC, Richardson JC, Olszewski J. Progressive supranuclear palsy. A heterogeneous degeneration involving the brain stem, basal ganglia and cerebellum with vertical gaze and pseudobulbar palsy, nuchal dystonia and dementia. Arch Neurol 1964;10:333-59.
Golbe LI, Davis PH, Schoenberg BS, Duvoisin RC. Prevalence and natural history of progressive supranuclear palsy. Neurology 1988;38:1031-4.
Litvan I, Agid Y, Calne D, Campbell G, Dubois B, Duvoisin RC, et al
. Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome): Report of NINDS-SPSP International workshop. Neurology 1996;47:1-9.
Groschel K, Kastrup A, Litvan I, Schulz JB. Penguins and hummingbirds: midbrain atrophy in progressive supranuclear palsy. Neurology 2006;66:949-50.
Righini A, Antonini A, De Notaris R, Bianchini E, Meucci N, Sacilotto G, et al
. MR imaging of the superior profile of the midbrain: Differential diagnosis between progressive supranuclear palsy and Parkinson disease. AJNR Am J Neuroradiol 2004;25:927-32.
Litvan I, Campbell G, Mangone CA, Verny M, McKee A, Chaudhuri KR, et al
. Which clinical features differentiate progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) from related disorders? A clinicopathological study. Brain 1997;120:65-74.
Nijboer H, Dautzenberg PL. Progressive supranuclear palsy: Acetylcholineeserase-inhibitor a possible therapy. Tijdschr Gerontol Geriatr 2009;40:133-7.
Liepelt I, Gaenslen A, Godau J, Di Santo A, Schweitzer KJ, Gasser T, et al
. Rivastigmine for the treatment of dementia in patients with progressive supranuclear palsy: Clinical observations as a basis for power calculations and safety analysis. Alzheimer′s Dement 2010;6:70-4.
Abe K. Zolpidem therapy for movement disorders. Recent Pat CNS Drug Discov 2008;3:55-60.
[Figure 1], [Figure 2], [Figure 3]