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nizam

Re: What is your diagnosis?

Dear Dr Iqbal,

You got the right diagnosis and thanks for the literature review. In the last 4 years I have seen 3 such cases. 2 responded very well to epidural blood patch and the third is currently hospitalized.

Post LP orthostatic headaches also respond dramatically to lumbar epidural blood patches.

Nizam

iqbalmd

Re: What is your diagnosis?

Thank you for continued contributions to this forum.
Great Case, Please if you can pose a case question every week that will be great as your cases and input is always invaluebale.


SIH or Spontaneous Intracranial Hypotension

Spontaneous intracranial hypotension (SIH) is often misdiagnosed, causing significant delay in treatment and risks of mistreatment, according to the results of a study published in the December issue of the Archives of Neurology.

"Spontaneous intracranial hypotension is an important cause of 'new daily persistent headaches' but is not a well-recognized entity," writes Wouter I. Schievink, MD, from the Cedars-Sinai Medical Center in Los Angeles, California. "The misdiagnosis of SIH can have serious consequences."

From Jan. 1, 2001, through June 30, 2002, 18 consecutive patients with SIH were evaluated for definitive surgical treatment of spontaneous spinal cerebrospinal fluid (CSF) leak. The initial diagnosis was incorrect in 17 patients (94%), with a diagnostic delay ranging from four days to 13 years (median, five weeks).

Migraine, meningitis, and psychogenic disorder were the most common misdiagnoses. Procedures performed for conditions mimicking SIH included cerebral arteriography in two patients, craniotomy for Chiari malformation in two patients and for evacuation of subdural hematoma in one patient, and brain biopsy in one patient.

"The patients in this report were well educated, had medical insurance with ready access to medical care, and had symptoms severe enough to warrant consideration of surgical correction of the underlying spinal CSF leak and thus were more likely to be diagnosed," Dr. Schievink writes. "It is likely that for other patients with SIH, the diagnostic delay is even more pronounced, or the correct diagnosis is never established."

The management of SIH includes bed rest, oral hydration, caffeine, and steroids, with placement of one or more lumbar epidural blood patches, followed by directed epidural blood patch or percutaneous placement of fibrin glue if there is still no relief.

"Surgical treatment is reserved for those patients in whom these nonsurgical measures have failed," according to Dr. Schievink.

Arch Neurol. 2003;60:1713-1718


Clinical Context
SIH has been recognized as an entity for more than six decades, and magnetic resonance imaging (MRI) has taken over from lumbar puncture as the diagnostic method of choice, according to a study by Chung and colleagues in the Nov. 14, 2000, issue of Neurology. Cerebral spinal fluid leak due to dural dents or fragile arachnoid cysts, in the presence of a previously unrecognized condition such as connective tissue disorder, is the most likely cause. Headache, and particularly positional headache, is the most common presenting symptom, thus patients may consult with a primary care physician, emergency physician, or a neurologist at the first presentation.

This is a descriptive retrospective case series report based on 18 patients after the diagnosis of SIH was made.

Study Highlights
15 women and 3 men, aged 22 to 55 years (mean age, 38 years) with health insurance and a high education level, were identified over 1.5 years and confirmed by computed tomographic myelography to have had a spontaneous spinal CSF leak. 12 patients were white and the remaining patients were black, Hispanic, or Asian.
Retrospective interviews with patients and their physicians, by telephone or in writing, and medical record review were used to establish the disease course and management.
One patient was correctly diagnosed, while 17 patients received incorrect diagnoses and sought attention from 1 to 6 physicians each before the correct diagnosis was made.
A total of 33 working diagnoses were generated on the 17 patients initially incorrectly diagnosed.
Diagnostic procedures on the 17 patients included cerebral arteriography in 2 patients, craniotomy with meningeal biopsy in 1 patient, and transesophageal echocardiography in 1 patient.
The diagnostic delay ranged from 4 days to 13 years (median, 5 weeks; mean, 13 months).
Incorrect treatments included craniotomies for decompression of cerebellar tonsillar descent in 2 patients and evacuation of subdural fluid collection in 1 patient.
Correct subsequent treatment of the 18 patients consisted of epidural blood patching in 5 patients and surgical repair of the spinal leak in 13 patients.
Headache was the most common presenting symptom. It typically worsened after patients assumed an upright position and improved in the recumbent position. Onset of headache was usually gradual, reaching maximum intensity in minutes to hours, but some patients reported acute onset of headaches ("thunderclap" headache) leading to the incorrect diagnosis of subarachnoid hemorrhage.
Other symptoms associated with SIH included nausea, vomiting, photophobia, and posterior neck pain or stiffness. Less common symptoms were facial numbness or pain (trigeminal nerve), weakness (facial nerve), and dysgeusia (chorda tympani). Transient visual symptoms included blurring and field defects, which may have been due to stretching of the optic apparatus over the pituitary fossa. Cervical nerve root stretching may result in radicular arm pain or numbness.
Very rarely, severe sagging of the brain may lead to coma from hind brain herniation. Other rare manifestations include parkinsonism, dementia, and cognitive defects.
MRI characteristically showed enhancement of the pachymeninges, downward displacement of the brain, and subdural fluid collections.
Management consists of bed rest, increased oral fluids, caffeine, and glucocorticoid and mineralcorticoid therapy as initial medical approaches. Persistent SIH is treated with placement of 1 or more lumbar epidural blood patches using 10 to 15 mL of blood. A high volume (20-40 mL) blood patch is used for cervical or thoracic spine leaks. Fibrin glue may be used if the blood patch fails.
Surgical repair of the leak is used only after medical measures fail.
Pearls for Practice
Most patients with SIH are incorrectly diagnosed with significant delay in correct diagnosis.
Headache, particularly one that worsens with upright posture, is the most common presenting symptom, and SIH should be considered as the diagnosis by physicians who care for patients with headache.




[Edited by iqbalmd on 05-11-2006 at 07:26 PM GMT]

[Edited by iqbalmd on 05-11-2006 at 07:28 PM GMT]

nizam

What is your diagnosis?

A 43-year-old woman presented with a relatively sudden onset of severe headache. Her headaches significantly improved on lying down. There was a reproducible severe excruciating throbbing headache with nausea and vomitting each time she sat-up or assumed an upright position. Headaches would completely resolve within 15-20 minutes of lying flat. A CT scan of the head showed bilateral small subdural hematomas but no sign of subarachnoid blood Her exam was normal with no papiledema or signs of meningismus. MRI of the brain with gadolinium (contrast) showed meningeal enhancement. Spinal tap was done after the MRI and was absolutely normal. Based on this clinical picture and CT and MRI findings a diagnosis was suspected and a procedure performed. Patients headaches completely resolved and she was discharged home. Please note that the subdural hematomas were not the cause but a consequence of her underlying condition.

What is your diagnosis? And, how do you treat?