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.

Idiopathic hypertrophic pachymeningitis

 

  Dumont AS, Clark AW, Sevick RJ, Myles ST. Idiopathic hypertrophic pachymeningitis:  A report of two cases and review of the literature.

 

Background-- Authors note entity was described by Charcot and Joffroy, and that there are three forms:  spinal, intracranial and craniospinal (latter is rarer). 

 

Past cases were often attributed to specific etiologies but most recent cases are idiopathic after investigation.  Authors case 1 underwent 2 surgeries for biopsy/decompression , had persistent pain and numbness, but was non progressive for 15 years after one early relapse.  The second one received steroids after biopsy with resolution of symptoms and MRI changes.  Authors argue based on above that the condition is not autoimmune. 

 

Literature review suggested a worse prognosis for patients with "inflammatory signs" (fever, high sed rate, CRP, or elevated WBC). 

 

Associations include infections: syphilis, TB, HTLV-1, fungi; may be presenting sign of adjacent ear or sinus infection.  Autoimmune associations include RA, orbital pseudotumor, multifocal fibrosclerosis, MCTD, Wegener's granulomatosis.    The 23 reports run the gamut from marked to slight improvement to deterioration and late recurrence or surgical death. 

 

For interest, Charcot's clinical descriptors divided the spinal form into distinct stages: first intermittent radicular pain that later became continuous; then muscle weakness and atrophy; then spastic paralysis and loss of sphincter control.  Radicular signs can be confined to the upper extremities and evolve over weeks to months or even a year. 

 

The cranial form frequently presents with a headache, cranial neuropathies and ataxia. 

 

Authors emphasize the need for pathologic confirmation.

CNS metastases in breast cancer patients: prognostic implications of tumor subty

CNS metastases in breast cancer patients: prognostic implications of tumor subtype; Bachmann C, Schmidt S, Staebler A, Fehm T, Fend F, Schittenhelm J, Wallwiener D, Grischke E; Medical Oncology (Northwood; London; England) 32 (1), 400 (Jan 2015)

 

Development of brain metastases (BM) in breast cancer leads to limited survival. The therapeutical options are limited. There are less data about the risk factors and prognostic importance in BM. Objective is to investigate predictors of central nervous system metastases and outcome after diagnosis of BM according to tumor subtype. Based on medical records, 80 consecutive patients with primary non-metastatic operable breast cancer, treated at Department of Gynecology, University of Tübingen, and who developed BM during follow-up, were retrospectively analyzed. Clinicopathological parameters and their prognostic impact were evaluated. A node involvement (40 %), ER/PR negative (53.75 vs. 61.25 %), triple negative (28.75 %) and HER2+ status (40 %) were associated with BM. BM in breast cancer patients lead to a shortened survival. In cerebral metastatic breast cancer patients with HER2-negative and triple-negative, patients had significant shorter survival after detection of BM compared with HER2-positive and non-triple-negative patients (p = 0.001; p = 0.03). Risk of BM varies significantly by subtype. Understanding the biology of metastases can help categorize patients into prognostically useful categories and tailor treatment regimens for individual patients. Prospective clinical trials would be required for evaluating the potential role of screening for asymptomatic BM and of treatment of triple-negative patients.

MMN and treatment responsiveness

 

Slee M, Selvan A, Donaghy M.  Neurology 2007; 69:1680-1687.

 

Authors look at 47 patients retrospectively and found that strict adherence to consensus criteria was a barrier to treatment.  Neither conduction block nor antibody status is a reliable predictor of treatment responsiveness.

 

Some of the pearls in the article:  Conduction block was present in 66%; GM1 antibody in a minority (25 %) and was not associated with conduction block. 27 % had lower limb onset, bu tin those patients, arm weakness typically developed subsequently.  Patients may have an acute or subacute onset with spontaneous resolution.  MMN can be PRECIPITATED by the use of steroids, and is usually not helped by steroids.  It "is evident from our study and others (Pakkiam AS, Perry GJ, Multifocal motor neuropathy without overt conduction block. Muscle Nerve 1998; 21: 243-245) that IVIG responsive  MMN occurs regularly without conduction block being demonstrated."  "A redefinition of conduction block which eliminates temporal dispersion as a restrictive factor and which helps predict IVIG responsiveness, (see Ghosh and Busby, JNNP 2005) categorized a further 15 % of our patients with conduction block."  "Our study suggests a decrement if IVIG responsiveness over time."  Using higher doses of 1.92 g/kg/6 weeks does better than Utrecht group of .54 g/kg /mo.  Disability self reports were more accurate than MRC scale scores with overall clinical change.

 

"Recognition of the clinical picture is the mainstay of diagnosis of MMN outside the researchg setting.  Weakness in nonwasted muscles and differential weakness across a common terminal motor nerve are the cardinal features.  Differential finger extension weakness is a frequent early manifestation likely reflecting vulnerabilities of the terminal branches of the posterior interosseous nerve." 

 

Blogger note-- this citation is extremely important for dealing with insurance companies to obtain approval for IVIG. 

 

Take away-- to take care of the patient, don't look at the test, be a doctor and a neurologist

Diploma?

Diploma?

http://tiny.cc/txntso23yf

Causes of pappilledema (bilateral) with good optic nerve function

increased intracranial pressure

 

malignant hypertension

 

toxins (amiodarone,cyclosporine)

 

sleep apnea syndrome

 

uveitis, eg. sarcoidosis

 

Abdominal auras and left sided localization of cps

Abdominal auras in patients with mesial temporal sclerosis; Kuan YC, Shih YH, Chen C, Yu HY, Yiu CH, Lin YY, Kwan SY, Yen DJ; Epilepsy & Behavior 25 (3), 386-390 (Oct 2012)To better clarify abdominal auras and their clinical correlates, we enrolled 331 temporal lobe epilepsy patients who received surgical treatment. Detailed descriptions of their auras were obtained before surgery and reconfirmed during postoperative outpatient follow-ups. Pathology revealed mesial temporal sclerosis (MTS) in 256 patients (77.3%) and 75 non-MTS. Of 214 MTS patients with auras, 78 (36.4%) reported abdominal auras (vs. 30.4% in non-MTS, p=0.439): 42 with left-sided seizure onset, and 36 with right-sided seizure onset. Moreover, 49 of the 78 MTS patients had abdominal auras accompanied by rising sensations (vs. 2 of 14 in non-MTS group, p=0.004). The"rising air"was initially described to locate to the epigastric (47.8%) or periumbilical area (45.7%) and mostly reached the chest (40.4%) or remained in the abdominal region (27.1%). An epigastric location of"rising air"favored a left-sided seizure onset, and non-epigastric areas favored right-sided seizure onset (p=0.018). Finally, we found that abdominal auras with or without rising sensations did not predict postoperative seizure outcomes.