Malignant subtype of Parkinsons

JAMA Neurology august 2015

Importance  There is increasing evidence that Parkinson disease (PD) is heterogeneous in its clinical presentation and prognosis. Defining subtypes of PD is needed to better understand underlying mechanisms, predict disease course, and eventually design more efficient personalized management strategies.

Objectives  To identify clinical subtypes of PD, compare the prognosis and progression rate between PD phenotypes, and compare the ability to predict prognosis in our subtypes and those from previously published clustering solutions.

Design, Setting, and Participants  Prospective cohort study. The cohorts were from 2 movement disorders clinics in Montreal, Quebec, Canada (patients were enrolled during the period from 2005 to 2013). A total of 113 patients with idiopathic PD were enrolled. A comprehensive spectrum of motor and nonmotor features (motor severity, motor complications, motor subtypes, quantitative motor tests, autonomic and psychiatric manifestations, olfaction, color vision, sleep parameters, and neurocognitive testing) were assessed at baseline. After a mean follow-up time of 4.5 years, 76 patients were reassessed. In addition to reanalysis of baseline variables, a global composite outcome was created by merging standardized scores for motor symptoms, motor signs, cognitive function, and other nonmotor manifestations.

Main Outcomes and Measures  Changes in the quintiles of the global composite outcome and its components were compared between different subtypes.

Results  The best cluster solution found was based on orthostatic hypotension, mild cognitive impairment, rapid eye movement sleep behavior disorder (RBD), depression, anxiety, and Unified Parkinson's Disease Rating Scale Part II and Part III scores at baseline. Three subtypes were defined as mainly motor/slow progression, diffuse/malignant, and intermediate. Despite similar age and disease duration, patients with the diffuse/malignant phenotype were more likely to have mild cognitive impairment, orthostatic hypotension, and RBD at baseline, and at prospective follow-up, they showed a more rapid progression in cognition (odds ratio [OR], 8.7 [95% CI, 4.0-18.7]; P < .001), other nonmotor symptoms (OR, 10.0 [95% CI, 4.3-23.2]; P < .001), motor signs (OR, 4.1 [95% CI, 1.8-9.1]; P = .001), motor symptoms (OR, 2.9 [95% CI, 1.3-6.2]; P < .01), and the global composite outcome (OR, 8.0 [95% CI, 3.7-17.7]; P < .001).

Conclusions and Relevance  It is recommended to screen patients with PD for mild cognitive impairment, orthostatic hypotension, and RBD even at baseline visits. These nonmotor features identify a diffuse/malignant subgroup of patients with PD for whom the most rapid progression rate could be expected.

View Original Article

Pellagra and spinal myoclonus

 

Park K, Oeda T, Sawada H. A case of alcoholic pellegra encephalopathy presenting with spinal myoclonus.  Neurology Clinical Practice 5; 472-3.

 

The authors present a case of alcoholic pellagra  with confusion and myoclonus responding dramatically to administration of niacin1500 mg per day starting 16 days after admission.  Essential points include:

 

1. Pellagra is rare in US but not in alcoholics

2. Dermatitis may be subtle and not appreciated

3.  Thiamine and niacin levels may be normal

4.  Thiamine may cause worsening due to increased demand for niacin

5.  Myoclonus in context is important to diagnosis, often stimulus sensitive

6.  Severe sensory ataxia, incontinence and dysautonomia also occur and improve with treatment

 

the 4 D's of pellagra, again, are , diarrhea, dementia, dermatitis and death

Scurvy in neurologic disease

 

Meisal K, Daggubati S,Josephson SA. .  Scurvy in the 21st century?  Vitamin C deficiency presenting to the neurologist.  Neurol Clin Prac 2015; 5:491-493.

 

Authors present a series of cases with vitamin C deficiency and review some of the neuro manifestations and non neuro manifestations, ; the former are not widely known. 

 

Patients with deficiency were caused by various other causes,including autism, poor status without access to produce, usually rural, were not alcohol users, had measurable low vitamin C levels.  Gingival hyperplasia, rash and bleeding were non neurologic manifestations. People bruised,especially on their thighs, Some had other nutritional diseases..  Pain, achiness and weight loss are expected.

 

Neuro manifestations included positional tremor, neuralgias100 %), focal weakness (50 %)  including footdrop and scapular winging, normal MRI's, long tract signs including hyperreflexia and plantar extensors, fatigue, trouble concentrating, headache, anxiety, and imbalance.

 

Patients recovered dramatically with treatment.