Monday, July 28, 2008

Misc. Signs of drug use/withdrawal

1.  tachycardia
2.  hyperthermia
3.  purulent drainage from nares with erosions (think cocaine)
4.  pop marks on skin
5.  hear murmur/ other signs of SBE (see Harrison's textbook for list)

Differential of delirium with quadriparesis/paraparesis

1.  central pontine myelinolysis
2.  progressive multifocal leukoencephalopathy
3.  acute disseminated encephalomyelitis
4.  Cobalamin deficiency (have not seen like this)
5.  cryptococcal meningitis
6.  critical illness neuromyopathy
7.  new onset myasthenia gravis, unsuspected, postoperative

Wednesday, July 16, 2008

Porphyria pearls

taken from NEJM CPC 2008; 358;2813-2825

case (abbreviated severely). 57 year old woman was admitted with abdominal pain and weakness. She had just had bariatric surgery for obesity. MRI showed changes of posterior reversible leuokoencephalopathy syndrome (turns out it occurs in porphyria). She had had intermittent dark urine and tachycardia.

Patient developed numbness, weakness and tingling in lower extremities, fell while walking and had an episode of confusion and urinary incontinence. She quickly (within days) could not stand or sit independently, became incontinent of stool with numbness and tingling along her body. She had 4/5 strength throughout, with fatigueing and absent reflexes. Sensation was normal. CSF showed normal glucose, protein and no cells. EMG showed an acute motor neuropathy. Strength deteriorated to 0-3/5, worse in proximal than distal legs. There was no response to IVIG.

Pearls-- factors for porphyria in this case were recurrent severe abdominal pain, dark or reddish urine, arterial hypertension, tachycardia and constipation. The diagnostic test in 5-ALA and PBG in urine or serum. A rapid test is the PBG in urine (Hoesch or Watson-Schwartz reaction). Key is diagnose porphyria first then type it. (False positives in 5-ALA are lead posioning and hereditary tyrosinemia type I).

Most types are inherited but the most common type, porphyria cutanea tarda is acquired, associated with liver disease and iron overload. Of 4 types, any may present with neurovisceral presentation esp colicky abdominal pain, and hereditary coproporphyria and variegate porphyria can also present with cutaneous features. Attacks are likely precipitated by adverse effects of excess ALA which is structurally similar to GABA. Starvation, poor CH2O/ energy intake, drugs, alcohol, smoking, infections and stress can ppt. In this case the negative energy balance with surgery caused up regulation of hepatic ALA synthase 1, due to loss of CH2O repression of rate controlling enzyme for heme synthesis in the liver. Other "bad " drugs in this case were phenytoin, tramadol and bactrim. Sulfonamides and barbiturates are also "bad." So is progesterone (which is why postpubertal women are more susceptible). The treatment is i-v heme. It can prevent reversible axonal death. Prognosis is slow and incomplete recovery. Noted also in this case wwre blisters in sun exposed parts of the body. She had variegate porphyria.

Monday, July 14, 2008

MoCa test for cognitive impairment

The MoCA test is an alternative to the MMSE which has had its share of problems.  Link for information

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Friday, July 11, 2008

Insulinoma mimicking seizure

Bazil CW, Pack A Insulinoma presenting as a seizure disorder. Neurology 2001; 56:817-818.

This rare disease mimicking a common disease needs to be added to the knowledge of doctors diagnosing individuals with various "spells." A 45 year old woman had troubel with panic attacks, inability to focus, tongue/limb tingling, perception of yellow spots and cold sweat. She later developed episodic unresponsiveness and jerking. Waxing and waning irda and polymorphic delta was seen on eeg, without interictal discharges, and the patient was felt to have pseudoseizures. A low glucose of 55 was found, islet cell tumors of the pancrease were found and removed and the patient took diazoxide with improvement.

Insulinoma usually includes nonspecific symptoms such as dizziness, apathy, headache and confusion. It is rare. affecting at most 5 per million.

Neuropsychiatric Profiles of Patients with Insulinomas; Ding Y, Wang S, Liu J, Yang Y, Liu Z, Li J, Zhang B, Chen Y, Ding M; European Neurology 63 (1), 48-51 (Dec 2009)
A review of 42 patients with insulinoma was undertaken, showing that many went undiagnosed for up to five years with neurologic and neuropsychiatric symptoms. 12 were misdiagnosed as epilepsy with eeg findings. Others received psychiatric diagnoses. Clinical presentation was usually confusion, convulsions and visual disturbance.